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Farber Lipogranulomatosis
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Definition
: A sphingolipidosis subtype that is characterized by the histological appearance of granulomatous deposits in tissues. It results from the accumulation of CERAMIDES in various tissues due to an inherited deficiency of ACID CERAMIDASE.
Other names
Lipogranulomatosis, Farber; Deficiency, Acid Ceramidase; Ceramidase Deficiency, Acid; Acid Ceramidase Deficiencies; Farber Disease; Acid Ceramidase Deficiency
Substance
CAS Registry & name
Categories
Source
Farber's disease
0
*Farber Lipogranulomatosis.
ASAH1 protein, human
EC 3.5.1.23
*Acid Ceramidase
Farber Lipogranulomatosis.