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Serum insulin-like system alterations in patients with spinocerebellar ataxia type 3.
- Source :
-
Movement disorders : official journal of the Movement Disorder Society [Mov Disord] 2011 Mar; Vol. 26 (4), pp. 731-5. Date of Electronic Publication: 2010 Nov 10. - Publication Year :
- 2011
-
Abstract
- Spinocerebellar ataxias (SCAs) constitute a group of autosomal dominant neurodegenerative disorders with no current treatment. The insulin/insulin-like growth factor 1 (IGF-1) system (IIS) has been shown to play a role in the neurological dysfunction of SCAs and other polyglutamine disorders. We aimed to study the biomarker profile of serum IIS components in SCA3. We performed a case-control study with 46 SCA3 patients and 42 healthy individuals evaluating the peripheral IIS profile (insulin, IGF-1, IGFBP1 and 3) and the correlation with clinical, molecular, and neuroimaging findings. SCA3 patients presented lower insulin and IGFBP3 levels and higher insulin sensitivity (HOMA2), free IGF-I, and IGFBP1 levels when compared with controls. IGFBP-1 levels were directly associated with CAG expanded repeat length; IGF-1 was associated with the volumetries of specific brainstem regions on magnetic resonance imaging (MRI). Insulin levels and sensitivity were related to age at onset of symptoms. Our findings indicate an involvement of IIS components in SCA3 neurobiology and IGFBP-1 as a potential biomarker of the disease.<br /> (Copyright © 2010 Movement Disorder Society.)
- Subjects :
- Adult
Ataxin-3
Case-Control Studies
Female
Humans
Insulin-Like Growth Factor Binding Protein 3
Machado-Joseph Disease genetics
Male
Middle Aged
Nerve Tissue Proteins genetics
Nuclear Proteins genetics
Repressor Proteins genetics
Insulin blood
Insulin-Like Growth Factor Binding Protein 1 blood
Insulin-Like Growth Factor Binding Proteins blood
Insulin-Like Growth Factor I metabolism
Machado-Joseph Disease blood
Subjects
Details
- Language :
- English
- ISSN :
- 1531-8257
- Volume :
- 26
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Movement disorders : official journal of the Movement Disorder Society
- Publication Type :
- Academic Journal
- Accession number :
- 21506152
- Full Text :
- https://doi.org/10.1002/mds.23428