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Genetic alterations influence the malignant potential of cancer cells, and so does the tumor microenvironment. Herein, we combined the study of KRAS oncogenic effects in colorectal cancer cells with the influence of fibroblast-derived factors. Results revealed that mutant KRAS regulates cell fate through both autonomous and nonautonomous signaling mechanisms. Specifically, processes such as proliferation and cell-cell aggregation were autonomously controlled by mutant KRAS independently of the stimulation with fibroblasts conditioned media. However, cancer cell invasion revealed to be a KRAS-dependent nonautonomous effect, resulting from the cooperation between fibroblast-derived HGF and mutant KRAS regulation of C-MET expression. C-MET downregulation upon KRAS silencing rendered cells less responsive to HGF and thus less invasive. Yet, in one cell line, KRAS inhibition triggered invasion upon stimulation with fibroblasts conditioned media. Inhibition of PIK3CA oncogene did not promote invasion, thus showing a KRAS-specific effect. Moreover, the invasive capacity also depended on the HGF-C-MET axis. Overall, our study awards oncogenic KRAS an important role in modulating the response to fibroblast-secreted factors either by promoting or impairing invasion, and depicts the HGF-C-MET axis as a putative therapeutic target to impair the invasive properties of mutant KRAS cancer cells.

The quality and safety of health care is a priority, a requirement and a demand of health organizations and social institutions with concrete purposes of progressively providing people with a higher level of health and well-being. It is in the development of this path that home care currently represents an area of gradual investment and where health care services and the scientific community have shown interest in building circuits and instruments that can respond to needs. It is essencial that care must be centered and in close proximity to the person and their family, their context. On the other hand, in Portugal, there are already quality and safety models for the institutionalization context however it is non-existent for home care. In this sense, our objective is to identify, through a systematic review of the literature, particularly from the last 5 years, areas of quality and safety in home care.

Cilia are involved in a plethora of motility and sensory-related functions. Ciliary defects cause several ciliopathies, some of which with late-onset, suggesting cilia are actively maintained. While much is known about cilia assembly, little is understood about the mechanisms of their maintenance. Given that intraflagellar transport (IFT) is essential for cilium assembly, we investigated the role of one of its main players, IFT88, in ciliary maintenance. We show that DmIFT88, the Drosophila melanogaster orthologue of IFT88, continues to move along fully formed sensory cilia, and that its acute knockdown in the ciliated neurons of the adult affects sensory behaviour. We further identify DmGucy2d, the Drosophila guanylyl cyclase 2d, as a DmIFT88 cargo, whose loss also leads to defects in sensory behaviour maintenance. DmIFT88 binds to the intracellular part of DmGucy2d, a highly, evolutionarily conserved and mutated in several degenerative retina diseases, taking the cyclase into the cilia. Our results offer a novel mechanism for the maintenance of sensory cilia function and its potential role in human diseases.

Cilia are involved in a plethora of motility and sensory-related functions. Ciliary defects cause several ciliopathies, some of which with late-onset, suggesting cilia are actively maintained. While much is known about cilia assembly, little is understood about the mechanisms of their maintenance. Given that intraflagellar transport (IFT) is essential for cilium assembly, we investigated the role of one of its main players, IFT88, in ciliary maintenance. We show that DmIFT88, the Drosophila melanogaster orthologue of IFT88, continues to move along fully formed sensory cilia, and that its acute knockdown in the ciliated neurons of the adult affects sensory behaviour. We further identify DmGucy2d, the Drosophila guanylyl cyclase 2d, as a DmIFT88 cargo, whose loss also leads to defects in sensory behaviour maintenance. DmIFT88 binds to the intracellular part of DmGucy2d, a highly, evolutionarily conserved and mutated in several degenerative retina diseases, taking the cyclase into the cilia. Our results offer a novel mechanism for the maintenance of sensory cilia function and its potential role in human diseases.

Cancers are complex tissues composed by genetically altered cancer cells and stromal elements such as inflammatory/immune cells, fibroblasts, endothelial cells and pericytes, neuronal cells, and a non-cellular component, the extracellular matrix. The complex network of interactions and crosstalk established between cancer cells and the supportig cellular and non-cellular components of the microenvironment are of extreme importance for tumor initiation and progression, strongly impacting the course and the outcome of the disease. Therefore, a better understanding of the tumorigenic processes implies the combined study of the cancer cell and the biologic, chemical and mechanic constituents of the tumor microenvironment, as their concerted action plays a major role in the carcinogenic pathway and is a key determinant of the efficacy of anti-cancer treatments. The use of animal models (e.g. Mouse, Zebrafish and Drosophila) to study cancer has greatly impacted our understanding of the processes governing initiation, progression and metastasis and allowed the discovery and pre-clinical validation of novel cancer treatments as it allows to recreate tumor development in a more pathophysiologic environment.

Incontinence-associated dermatitis (IAD) it is a common complication in people with loss of continence sphincters, which induces irritation and disruption of the skin, with impact on quality of life and increase vulnerability to pressure ulcers and secondary infections. Objective: Identify which nursing interventions aimed at the prevention and healing of IAD in acute and long-term care. Method: We conducted a retrospective survey between January 2010 and September 2016 by formulating a question in PI[C]O format in databases MEDLINE (with full text) e CINAHL (with full text), and 9 articles having been selected for analysis of a total of 537. Results: The observation of the skin is the gold standard. IAD Prevention: cleanse the skin with products with pH acid (step 1); apply emollients/ moisturizers on intact skin (step 2); protect the skin with barrier products (step 3). IAD Healing: clean the skin (step 1); skin protection (step 2); exudate management (if severe IAD) (step 3). The identified interventions can be applied to other body regions affected by humidity. Conclusions: prevention, diagnosis and IAD healing is a nursing-sensitive indicators. Implications for the nursing profession: Nursing practice based on evidence, in a continuous improvement logic, contributed to the improvement of results in the incidence and prevalence of IAD.

The clinical reasoning strategies nursing that emerged from the study were: as the development of effective relationship with the patient and family involvement, application of negotiation and compromise skills, use of critical-reflective skills for selection of nursing interventions, intuition, recognition of similar situations and hypothesis testing. And as clinical reasoning criteria the nurse assesses the risk-benefit for patients, organizational needs and sources of information (11). This study helped identify some strategies and clinical reasoning criteria nurse who cares for patients in critical condition. However, more studies are needed to deepen knowledge about the clinical reasoning process by promo- ting best results from the autonomous intervention and nursing decision making.

Centrosomes are the major microtubule organising centres of animal cells. Deregulation in their number occurs in cancer and was shown to trigger tumorigenesis in mice. However, the incidence, consequence and origins of this abnormality are poorly understood. Here, we screened the NCI-60 panel of human cancer cell lines to systematically analyse centriole number and structure. Our screen shows that centriole amplification is widespread in cancer cell lines and highly prevalent in aggressive breast carcinomas. Moreover, we identify another recurrent feature of cancer cells: centriole size deregulation. Further experiments demonstrate that severe centriole over-elongation can promote amplification through both centriole fragmentation and ectopic procentriole formation. Furthermore, we show that overly long centrioles form over-active centrosomes that nucleate more microtubules, a known cause of invasiveness, and perturb chromosome segregation. Our screen establishes centriole amplification and size deregulation as recurrent features of cancer cells and identifies novel causes and consequences of those abnormalities., Cancer cells are characterised by abnormalities in the number of centrosomes and this phenotype is linked with tumorigenesis. Here the authors report centriole length deregulation in a subset of cancer cell lines and suggest a link with subsequent alterations in centriole numbers and chromosomal instability.

Cilia are evolutionarily conserved structures with many sensory and motility-related functions. The ciliary base, composed of the basal body and the transition zone, is critical for cilia assembly and function, but its contribution to cilia diversity remains unknown. Hence, we generated a high-resolution structural and biochemical atlas of the ciliary base of four functionally distinct neuronal and sperm cilia types within an organism, Drosophila melanogaster. We uncovered a common scaffold and diverse structures associated with different localization of 15 evolutionarily conserved components. Furthermore, CEP290 (also known as NPHP6) is involved in the formation of highly diverse transition zone links. In addition, the cartwheel components SAS6 and ANA2 (also known as STIL) have an underappreciated role in basal body elongation, which depends on BLD10 (also known as CEP135). The differential expression of these cartwheel components contributes to diversity in basal body length. Our results offer a plausible explanation to how mutations in conserved ciliary base components lead to tissue-specific diseases. info:eu-repo/semantics/publishedVersion

KRAS is one of the most frequently mutated oncogenes in cancer, being a potent initiator of tumorigenesis, a strong inductor of malignancy, and a predictive biomarker of response to therapy. Despite the large investment to understand the effects of KRAS activation in cancer cells, pharmacologic targeting of KRAS or its downstream effectors has not yet been successful at the clinical level. Recent studies are now describing new mechanisms of KRAS-induced tumorigenesis by analyzing its effects on the components of the tumor microenvironment. These studies revealed that the activation of KRAS on cancer cells extends to the surrounding microenvironment, affecting the properties and functions of its constituents. Herein, we discuss the most emergent perspectives on the relationship between KRAS-mutant cancer cells and their microenvironment components. Cancer Res; 78(1); 7–14. ©2017 AACR.

BACKGROUND AND OBJECTIVES: To outline best practices guidelines to control pain and dyspnea of cancer patients in an urgency setting. CONTENTS: PI[C]O question, with resource to EBSCO (Medline with Full Text, CINAHL, Plus with Full Text, British Nursing Index), retrospectively from September 2009 to 2014 and guidelines issued by reference entities: Oncology Nursing Society (2011), National Comprehensive Cancer Network (2011; 2014) and Cancer Care Ontario (2010), with a total of 15 articles. The first stage for adequate symptoms control is systematized evaluation. Pharmacological pain control should comply with the modified analgesic ladder of the World Health Organization, including titration, equianalgesia, opioid rotation, administration route, difficult to control painful conditions and adverse effects control. Oxygen therapy and noninvasive ventilation are control modalities of some situations of dyspnea, where the use of diuretics, bronchodilators, steroids, benzodiazepines and strong opioids are effective strategies. Non-pharmacological measures: psycho-emotional support, hypnosis, counseling/training/instruction, therapeutic adherence, music therapy, massage, relaxation techniques, telephone support, functional and respiratory reeducation equally improve health gains. CONCLUSION: Cancer pain and dyspnea control require comprehensive and multimodal approach. Implications for nursing practice: best practice guidelines developed based on scientific evidence may support clinical decision-making with better quality, safety and effectiveness. RESUMO JUSTIFICATIVA E OBJETIVOS: Delinear linhas orientadoras de boa prática no controle da dor e dispneia, de pacientes com doença oncológica em serviço de urgência. CONTEÚDO: Pergunta PI[C]O, com recurso à EBSCO (Medline with Full Text, CINAHL, Plus with Full Text, British Nursing Index), retrospectivamente de setembro de 2009 até 2014 e guidelines emanadas por entidades de referência: Oncology Nursing Society (2011), National Comprehensive Cancer Network (2011; 2014) e Cancer Care Ontario (2010), dos quais resultou um total de 15 artigos. A primeira etapa para um controle adequado de sintomas é uma apreciação sistematizada. O tratamento farmacológico da dor deve-se reger pela escada analgésica modificada da Organização Mundial da Saúde, com inclusão da titulação, equianalgesia, rotatividade de opioides, vias de administração, condições dolorosas de difícil tratamento e controle de efeitos adversos. A oxigenoterapia e ventilação não invasiva são modalidades de controle de algumas situações de dispneia, onde a utilização de diuréticos, broncodilatadores, corticoides, benzodiazepínicos e opioides fortes são estratégias eficazes. As medidas não farmacológicas: apoio psicoemocional, hipnose, aconselhamento/treino/instrução, adesão terapêutica, musicoterapia, massagem, técnicas de relaxamento, apoio telefónico, reeducação funcional e respiratória aumentam igualmente os ganhos em saúde. CONCLUSÃO: O controle da dor oncológica e dispneia exigem uma abordagem compreensiva e multimodal. Implicações para a prática de Enfermagem: linhas orientadoras de boa prática, desenvolvidas com base na evidência científica podem suportar uma tomada de decisão clínica com maior qualidade, segurança e efetividade

Cilia are evolutionarily conserved structures with many sensory and motility-related functions. The ciliary base, composed of the basal body and the transition zone, is critical for cilia assembly and function, but its contribution to cilia diversity remains unknown. Hence, we generated a high-resolution structural and biochemical atlas of the ciliary base of four functionally distinct neuronal and sperm cilia types within an organism, Drosophila melanogaster. We uncovered a common scaffold and diverse structures associated with different localization of 15 evolutionarily conserved components. Furthermore, CEP290 (also known as NPHP6) is involved in the formation of highly diverse transition zone links. In addition, the cartwheel components SAS6 and ANA2 (also known as STIL) have an underappreciated role in basal body elongation, which depends on BLD10 (also known as CEP135). The differential expression of these cartwheel components contributes to diversity in basal body length. Our results offer a plausible explanation to how mutations in conserved ciliary base components lead to tissue-specific diseases.

Centrioles and cilia are highly conserved eukaryotic organelles. Drosophila melanogaster is a powerful genetic and cell biology model organism, extensively used to discover underlying mechanisms of centrosome and cilia biogenesis and function. Defects in centrosomes and cilia reduce fertility and affect different sensory functions, such as proprioception, olfaction, and hearing. The fly possesses a large diversity of ciliary structures and assembly modes, such as motile, immotile, and intraflagellar transport (IFT)-independent or IFT-dependent assembly. Moreover, all the diverse ciliated cells harbor centrioles at the base of the cilia, called basal bodies, making the fly an attractive model to better understand the biology of this organelle. This chapter describes protocols to visualize centrosomes and cilia by fluorescence and electron microscopy.

Centrioles and cilia are highly conserved eukaryotic organelles. Drosophila melanogaster is a powerful genetic and cell biology model organism, extensively used to discover underlying mechanisms of centrosome and cilia biogenesis and function. Defects in centrosomes and cilia reduce fertility and affect different sensory functions, such as proprioception, olfaction, and hearing. The fly possesses a large diversity of ciliary structures and assembly modes, such as motile, immotile, and intraflagellar transport (IFT)-independent or IFT-dependent assembly. Moreover, all the diverse ciliated cells harbor centrioles at the base of the cilia, called basal bodies, making the fly an attractive model to better understand the biology of this organelle. This chapter describes protocols to visualize centrosomes and cilia by fluorescence and electron microscopy.

Drosophila Rootletin organizes rootlets in sensory neurons, where it transmits multiple sensory inputs and maintains basal body cohesion, yet it is not required for cilium stability., Cilia are essential for cell signaling and sensory perception. In many cell types, a cytoskeletal structure called the ciliary rootlet links the cilium to the cell body. Previous studies indicated that rootlets support the long-term stability of some cilia. Here we report that Drosophila melanogaster Rootletin (Root), the sole orthologue of the mammalian paralogs Rootletin and C-Nap1, assembles into rootlets of diverse lengths among sensory neuron subtypes. Root mutant neurons lack rootlets and have dramatically impaired sensory function, resulting in behavior defects associated with mechanosensation and chemosensation. Root is required for cohesion of basal bodies, but the cilium structure appears normal in Root mutant neurons. We show, however, that normal rootlet assembly requires centrioles. The N terminus of Root contains a conserved domain and is essential for Root function in vivo. Ectopically expressed Root resides at the base of mother centrioles in spermatocytes and localizes asymmetrically to mother centrosomes in neuroblasts, both requiring Bld10, a basal body protein with varied functions.

Identificar estrategias de raciocinio clinico dos enfermeiros que cuidam de clientes, em si- tuacao critica. Foi realizada uma revisao sistematica da literatura com recurso ao metodo PI[C]O na EBSCO (CINAHL (Plus with Full Text), MEDLINE (Plus with Full Text)) com limi- tador temporal entre 2009-2015, dos quais foram selecionados 8 artigos. As estrategias de raciocinio clinico de enfermagem que emergiram foram: a relacao de con- fianca com o cliente e envolvimento da familia, aplicacao de competencias de negociacao e compromisso, de competencias critico-reflexivas, a intuicao, o reconhecimento de situa- coes similares e teste de hipoteses. E, como criterios de raciocinio clinico, o enfermeiro avalia o risco-beneficio para o cliente, avalia as necessidades organizacionais e pesquisa informacao em fontes complementares(11). Esta revisao permitiu conhecer algumas estrate- gias e criterios de raciocinio clinico do enfermeiro que cuida de clientes em situacao critica. No entanto, sao necessarios mais estudos para aprofundar conhecimento sobre o processo de raciocinio clinico que promove resultados positivos para o cliente decorrentes da inter- vencao autonoma e capacidade de decisao da enfermagem. Descritores: Raciocinio Clinico; tomada de decisao; cuidados de enfermagem. ABSTRACT Identify clinical reasoning strategies of nurses in critical care. A systematic review of the literature with the PI[C]O was held in the EBSCO (CINAHL (Plus with Full Text), MEDLINE (Plus with Full Text)), with time limit between 2009 – 2015, of which 8 articles were selected. The clinical reasoning strategies nursing that emerged from the study were: as the deve- lopment of effective relationship with the patient and family involvement, application of negotiation and compromise skills, use of critical-reflective skills for selection of nursing interventions, intuition, recognition of similar situations and hypothesis testing. And as clinical reasoning criteria the nurse assesses the risk-benefit for patients, organizational needs and sources of information(11). This study helped identify some strategies and clini- cal reasoning criteria nurse who cares for patients in critical condition. However, more studies are needed to deepen knowledge about the clinical reasoning process by promo- ting best results from the autonomous intervention and nursing decision making.Descriptors: Clinical Reasoning; decision making; nursing care.

A dermatite associada a incontinencia (DAI) trata-se de uma complicacao frequente nas pes- soas com perda de continencia de esfincteres, que induz irritacao e rotura da pele, com im- pacto na qualidade de vida e vulnerabilidade para ulceras por pressao e infecoes secundarias. Objetivo: Identificar quais as intervencoes de enfermagem dirigidas a prevencao e cica- trizacao da DAI, em cuidados agudos e de longa duracao. Metodologia: Efetuou-se uma pes- quisa retrospetiva entre janeiro de 2010 e setembro de 2016, mediante a formulacao de uma pergunta em formato PI[C]O, nas bases de dados: MEDLINE (with full text) e CINAHL (with full text), tendo sido selecionados 9 artigos para analise de um total de 537. Resultados: A observacao da pele e o gold standard . Prevencao de DAI: limpar a pele com produtos com pH acido ( step 1); aplicar emolientes/ hidratantes na pele integra ( step 2); proteger a pele com produtos barreira ( step 3). Cicatrizacao de DAI: limpar a pele ( step 1); protecao cutânea ( step 2); gestao de exsudado (se DAI severa) ( step 3). As intervencoes identificadas podem ser aplicadas em outras regioes corporais afetadas pela humidade. Conclusoes: A preven- cao, diagnostico e cicatrizacao de DAI e sensivel aos cuidados de enfermagem. Implicacoes para a profissao de enfermagem: A pratica de enfermagem baseada na evidencia, em uma logica de melhoria continua, contribuiu para a melhoria dos resultados ao nivel da inci- dencia e prevalencia da DAI. Palavras-chave: Dermatite Associada a Incontinencia, prevencao, cicatrizacao, interven- coes de enfermagem. Abstract Incontinence-associated dermatitis (IAD) it is a common complication in people with loss of continence sphincters, which induces irritation and disruption of the skin, with impact on quality of life and increase vulnerability to pressure ulcers and secondary infections. Objective: Identify which nursing interventions aimed at the prevention and healing of IAD in acute and long-term care. Method: We conducted a retrospective survey between January 2010 and September 2016 by formulating a question in PI[C]O format in databa- ses MEDLINE (with full text) e CINAHL (with full text), and 9 articles having been selected for analysis of a total of 537. Results: The observation of the skin is the gold standard. IAD Prevention: cleanse the skin with products with pH acid ( step 1); apply emollients/ mois- turizers on intact skin ( step 2); protect the skin with barrier products ( step 3). IAD Healing: clean the skin ( step 1); skin protection ( step 2); exudate management (if severe IAD) ( step 3). The identified interventions can be applied to other body regions affected by humidity. Conclusions: prevention, diagnosis and IAD healing is a nursing-sensitive indicators. Im- plications for the nursing profession: Nursing practice based on evidence, in a continuous improvement logic, contributed to the improvement of results in the incidence and preva- lence of IAD. Keywords: Incontinence-associated dermatitis, prevention, healing, nursing interventions.

Scandinavian Journal of Psychology, 2007, 48, 69–74 DOI: 10.1111/j.1467-9450.2006.00544.x © 2007 The Authors. Journal compilation © 2007 The Scandinavian Psychological Associations. Published by Blackwell Publishing L td., 9600 Garsington Road, Oxford OX4 2DQ, UK and 350 Main Street, Malden, MA 02148, USA. ISSN 0036-5564. Blackwell Publishing Ltd Health and Disability Semantic interference on a phonological task in illiterate subjects ALEXANDRA REIS, 1,2,3 L UÍS FAÍSCA, 2,3 SUSANA MENDONÇA, 2 MARTIN INGVAR 1 and KARL MAGNUS PETERSSON 1,3,4 1 Cognitive Neurophysiology Research Group, Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden 2 Cognitive Neuroscience Research Group, Departamento de Psicologia, Faculdade de Ciências Humanas e Sociais, Universidade do Alg arve, Faro, Por tugal 3 Center for Intelligent Systems, Universidade do Algarve, Faro, Portugal 4 F. C. Donders Centre for Cognitive Neuroimaging, Radboud University Nijmegen, The Netherlands R eis, A., Faísca, L., Mendonça, S., Ingvar, M. & Petersson, K. M. (2007). Semantic interference on a phonological task in illit erate subjects. Scandinavian Journal of Psychology , 48 , 69–74. Previous research suggests that learning an alphabetic written language influences aspects of the auditory-verbal language syste m. In this study, we e xamined whether literacy influences the notion of words as phonological units independent of lexical semantics in literate a nd illiterate subjects. Subjects had to decide which item in a word- or pseudoword pair was phonologically longest. By manipulating the relat ionship between r eferent size and phonological length in three word conditions (congruent, neutral, and incongruent) we could examine to what e xtent subjects f ocused on form rather than meaning of the stimulus material. Moreover, the pseudoword condition allowed us to examine global p honological aw areness independent of lexical semantics. The results showed that literate performed significantly better than illiterate subj ects in the neutral and incongruent word conditions as well as in the pseudoword condition. The illiterate group performed least well in the incong ruent condition and significantly better in the pseudoword condition compared to the neutral and incongruent word conditions and suggest that pe rformance on phonological word length comparisons is dependent on literacy. In addition, the results show that the illiter a te participant s are able to perceive and process phonological length, albeit less well than the literate subjects, when no semantic interference is present . In conclusion, the present results confirm and extend the finding that illiterate subjects are biased towards semantic-conceptual-pragmatic type s of cognitive processing EU grant QLK6-CT- 99-02140; Swedish Medical Research Council (8276); Knut and Alice Wallenberg Foundation; Swedish Dyslexia Association

A Formalina 10% Tamponada (FNT 10%) é considerada o fixador de eleição nos Laboratórios de Anatomia Patológica. Contudo, a exposição a esta substância acarreta riscos para a saúde dos técnicos. A International Agency for Research on Cancer (IARC) classificou-a como cancerígeno humano e estudos relevam uma correlação positiva entre a exposição a formaldeído e o desenvolvimento de leucemia e leucemia mielóide. Torna-se relevante alterar o processo de fixação substituindo a FNT 10% por outros fixadores melhorando estas questões. Como tal, desenvolveram-se fixadores à base de outros compostos químicos que não formaldeído, como o Fixador Molecular Universal (UMFIX) que tem como base metanol e polietilenoglicol e que é usualmente utilizado com um processador rápido de micro-ondas. Pretende-se comparar as diferenças entre a fixação por FNT 10% e a fixação por UMFIX, em tecido hepático processado em processador rápido de micro-ondas, para as colorações de Hematoxilina-Eosina (H&E), Tricrómio de Gomori, Reticulina e PAS, através da qualidade final das lâminas testadas. A coloração de H&E foi a única que apresentou diferenças estatisticamente significativas entre os dois fixadores (p=0,032; α

Cilia are microtubule-based membrane protrusions conserved across evolution involved in cell motility, fluid flow and sensing. The diversity in functions is generally attributed to a core conserved microtubule-based structure, the axoneme, decorated by different structures, membrane and signaling systems. Here we study four classes of cilia that represent very diverse motility and sensory functions within a single organism, the fruit fly. We uncover that the base of the cilia, the basal body and transition zone, is much more diverse than previously thought, showing large variation in number, length, ultrastructure, and connection to other cellular structures. We further demonstrate that basal body diversity is imparted by differential regulation of evolutionarily conserved core components. The tissue specific regulation of core basal body and transition zone genes suggests mechanisms that generate tissue specific phenotypes in human ciliopathic syndromes.