Document Detail


Genetic obesity syndromes.
MedLine Citation:
PMID:  18230893     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
There are numerous reports of multi-system genetic disorders with obesity. Many have a characteristic presentation and several, an overlapping phenotype indicating the likelihood of a shared common underlying mechanism or pathway. By understanding the genetic causes and functional perturbations of such syndromes we stand to gain tremendous insight into obesogenic pathways. In this review we focus particularly on Bardet-Biedl syndrome, whose molecular genetics and cell biology has been elucidated recently, and Prader-Willi syndrome, the commonest obesity syndrome due to loss of imprinted genes on 15q11-13. We also discuss highlights of other genetic obesity syndromes including Alstrom syndrome, Cohen syndrome, Albright's hereditary osteodystrophy (pseudohypoparathyroidism), Carpenter syndrome, MOMO syndrome, Rubinstein-Taybi syndrome, cases with deletions of 6q16, 1p36, 2q37 and 9q34, maternal uniparental disomy of chromosome 14, fragile X syndrome and Börjeson-Forssman-Lehman syndrome.
Authors:
Anthony P Goldstone; Philip L Beales
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Frontiers of hormone research     Volume:  36     ISSN:  0301-3073     ISO Abbreviation:  Front Horm Res     Publication Date:  2008  
Date Detail:
Created Date:  2008-01-30     Completed Date:  2008-05-07     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0320246     Medline TA:  Front Horm Res     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  37-60     Citation Subset:  IM    
Affiliation:
MRC Clinical Sciences Centre, Hammersmith Hospital, Imperial College London, UK. tony.goldstone@imperial.ac.uk
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MeSH Terms
Descriptor/Qualifier:
Acrocephalosyndactylia / genetics
Animals
Bardet-Biedl Syndrome / genetics
Brain / abnormalities
Chromosomes, Human, Pair 1 / genetics
Chromosomes, Human, Pair 14 / genetics
Chromosomes, Human, Pair 2 / genetics
Chromosomes, Human, Pair 6 / genetics
Chromosomes, Human, Pair 9 / genetics
Feeding Behavior
Fibrous Dysplasia, Polyostotic / genetics
Fragile X Syndrome / genetics
Gene Deletion
Humans
Obesity / genetics*
Prader-Willi Syndrome / genetics
Proteins / genetics
Rubinstein-Taybi Syndrome / genetics
Syndrome
Vesicular Transport Proteins / genetics
Chemical
Reg. No./Substance:
0/ALMS1 protein, human; 0/Proteins; 0/VPS13B protein, human; 0/Vesicular Transport Proteins

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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