- ►diabetesjournals.org CJA Smith, PA Crock, BR King, CJ Meldrum, RJ … - Diabetes Care, 2004 - Am Diabetes Assoc OBJECTIVE—Wolfram syndrome is an extremely rare autosomal-recessive disorder that predisposes
the development of type 1 diabetes in association with progressive optic atrophy. The genetic
basis of this disease has been shown to be due to mutations in the WFS1 gene. The ... Cited by 15 - Related articles - BL Direct - All 5 versions
SC McBain, NG Morgan - Biochemical and Biophysical Research …, 2003 - Elsevier Wolfram syndrome is a rare condition in which the pancreatic β-cells of patients are selectively
deleted during the early years of life by a non-autoimmune-mediated mechanism. The condition
is associated with mutations in the gene encoding wolframin, suggesting that this protein ... Cited by 8 - Related articles - All 5 versions
A Colosimo, V Guida, L Rigoli, C Di Bella, A De … - Human …, 2003 - interscience.wiley.com Wolfram syndrome (WS) is a recessively inherited mendelian form of diabetes and neurodegeneration
also known by the acronym DIDMOAD from the major clinical features, including diabetes
insipidus, diabetes mellitus, optic atrophy, and deafness. Affected individuals may also ... Cited by 12 - Related articles - BL Direct - All 4 versions
C Philbrook, E Fritz, H Weiher - Experimental gerontology, 2005 - Elsevier Wolfram or DIDMOAD Syndrome is clinically characterised by diabetes mellitus, diabetes
insipidus, optical atrophy and deafness (Fraser and Gunn, 1977 and Richardson and
Hamilton, 1977). Generally, diabetes mellitus appears in the first decade of life and ... Cited by 7 - Related articles - All 3 versions
- ►endojournals.org R Medlej, J Wasson, P Baz, S Azar, I Salti, J … - Journal of Clinical …, 2004 - Endocrine Soc In this study we describe 31 Lebanese WFS patients belonging to 17 families; this, to our
knowledge, is the largest number of patients reported in one series so far. Criteria for diagnosis
of WFS were the presence of insulin-dependent diabetes mellitus and optic atrophy ... Cited by 26 - Related articles - BL Direct - All 3 versions
F Giuliano, S Bannwarth, S Monnot, A Cano, B … - Human …, 2005 - interscience.wiley.com Wolfram syndrome (WS), a rare autosomal recessive neurodegenerative disorder, results in
most cases from mutations in the WFS1 gene. In this study, a total of 19 patients with Wolfram
syndrome and 36 relatives from 17 families were screened for mutations in the WFS1 ... Cited by 13 - Related articles - All 2 versions
E Domenech, M Gomez-Zaera, V Nunes - Clinical genetics, 2004 - interscience.wiley.com Wolfram syndrome (WS) is an autosomal recessive neurodegenerative disorder characterized
by early onset diabetes mellitus and progressive optic atrophy. Patients with WS frequently develop
deafness, diabetes insipidus, renal tract abnormalities, and diverse psychiatric illnesses, ... Cited by 10 - Related articles - BL Direct - All 4 versions
G Esteban Bueno, FM Gomez Trujillo - Revista Clinica Espanola, 2006 - cat.inist.fr Wolfram's syndrome(SW): diabetes mellitus(DM), diabetes insipidus(DI), blindness and
deafness, is multiorganic, hereditary and uncommon. Mitochondrial dysfunction damages the
oxidative pathway. Objective. To analyze the clinical characteristics, diagnostic delay in ... Cited by 1 - Related articles - All 3 versions
L Hansen, H Eiberg, T Barrett, T Bek, P … - European Journal of …, 2005 - nature.com Wolfram syndrome (WS) is a neuro-degenerative autosomal recessive (AR) disorder (OMIM
#222300) caused by mutations in the WFS1 gene on 4p16.1. More than 120 mutations have
been identified in WFS1 associated with AR WS, as well as autosomal dominant ... Cited by 10 - Related articles - BL Direct - All 6 versions
[CITATION] Wolfram syndrome: structural and functional analyses of mutant and wild-type …
