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Sjogren-Larsson Syndrome MeSH Descriptor Data 2022


MeSH Heading
Sjogren-Larsson Syndrome
Tree Number(s)
C16.131.831.512.723
C16.320.565.398.641.723
C16.320.850.820
C16.614.492.723
C17.800.428.333.723
C17.800.804.512.723
C17.800.827.820
C18.452.584.563.641.723
C18.452.648.398.641.723
Unique ID
D016111
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D016111
Annotation
do not confuse with SJOGREN'S SYNDROME; in titles & translations use diacritic: Sjögren-Larsson
Scope Note
An autosomal recessive neurocutaneous disorder characterized by severe ichthyosis MENTAL RETARDATION; SPASTIC PARAPLEGIA; and congenital ICHTHYOSIS. It is caused by mutation of gene encoding microsomal fatty ALDEHYDE DEHYDROGENASE leading to defect in fatty alcohol metabolism.
Entry Term(s)
Congenital Icthyosis Mental Retardation Spasticity Syndrome
FALDH Deficiency
Fatty Alcohol:NAD+ Oxidoreductase Deficiency
Fatty Aldehyde Dehydrogenase Deficiency
Fatty Aldehyde Dehydrogenase Deficiency Disease
Ichthyosis Oligophrenia Syndrome
Ichthyosis, Spastic Neurologic Disorder, and Oligophrenia
Sjögren-Larsson Syndrome
Previous Indexing
Ichthyosis (1966-1990)
Public MeSH Note
1991; see ICHTHYOSIS 1984-1990
History Note
1991; use ICHTHYOSIS 1984-1990
Date Established
1991/01/01
Date of Entry
1990/06/06
Revision Date
2021/07/01
Sjogren-Larsson Syndrome Preferred
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