TAY-SACHS

Pronunciation (US):

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 Dictionary entry overview: What does Tay-Sachs mean? 

• TAY-SACHS (noun)
  The noun TAY-SACHS has 1 sense:

1. a hereditary disorder of lipid metabolism occurring most frequently in individuals of Jewish descent in eastern Europe; accumulation of lipids in nervous tissue results in death in early childhood

  Familiarity information: TAY-SACHS used as a noun is very rare.

 Dictionary entry details 

• TAY-SACHS (noun)

Sense 1

Meaning:

A hereditary disorder of lipid metabolism occurring most frequently in individuals of Jewish descent in eastern Europe; accumulation of lipids in nervous tissue results in death in early childhood

Classified under:

Nouns denoting stable states of affairs

Synonyms:

infantile amaurotic idiocy; Sachs disease; Tay-Sachs; Tay-Sachs disease

Hypernyms ("Tay-Sachs" is a kind of...):

monogenic disease; monogenic disorder (an inherited disease controlled by a single pair of genes)

autosomal recessive defect; autosomal recessive disease (a disease caused by the presence of two recessive mutant genes on an autosome)

lipidosis (a disorder of lipid metabolism; abnormal levels of certain fats accumulate in the body)

 Context examples 

Some examples of genetic brain disorders include: • Leukodystrophies • Phenylketonuria • Tay-Sachs disease • Wilson disease

(Genetic Brain Disorders, NIH)

Representative examples include Gaucher disease, Tay-Sachs disease, and Niemann-Pick disease.

(Lipid Metabolism Disorder, NCI Thesaurus)

Mutations in at least 1 of 3 recessive genes: HEXA, HEXB, and GM2A cause defects in GM2 catabolism, leading to lysosomal lipid storage disorders that manifest primarily as neurodegenerative diseases, including Tay-Sachs and Sandhoff Disease.

(Ganglioside GM2, NCI Thesaurus)

Gangliosides are named as N-acetyl- (or N-glycoloyl-)neuraminosyl-(X)osylceramide, where (X) stands for the root name of the neutral oligosaccharide to which the sialosyl residue is attached ; found principally in nerve tissue, spleen, and thymus; GM1 accumulates in generalized gangliosidosis; GM2 accumulates in Tay-Sachs disease.

(Ganglioside, NCI Thesaurus)