ceramidase

ceramidase
An enzyme that hydrolyzes ceramides into sphingosine and a fatty acid; acylsphirgosine deacylase. A deficiency of this enzyme is associated with Farber disease.

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cer·am·i·dase (sə-ramґĭ-dās) [EC 3.5.1.23] an enzyme of the hydrolase class that catalyzes the cleavage of a ceramide (N-acylsphingosine) to form sphingosine and a fatty acid anion, a step in the degradation of sphingolipids. Acid, neutral, and alkaline isozymes occur; deficiency of the acid (lysosomal) enzyme, an autosomal recessive trait, results in accumulation of ceramides and gangliosides in Farber disease. Called also acylsphingosine deacylase.

Medical dictionary. 2011.

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  • Ceramidase — is an enzyme which cleaves fatty acids from ceramide, producing sphingosine (SPH) which in turn is phosphorylated by a sphingosine kinase to form sphingosine 1 phosphate (S1P).[1] Contents 1 Function 2 Clinical significance …   Wikipedia

  • Ceramidase-Mangel — Klassifikation nach ICD 10 E75.2 Sonstige Sphingolipidosen (inkl. Farber Krankheit) …   Deutsch Wikipedia

  • ceramidase — noun An enzyme that separates the two parts of ceramide …   Wiktionary

  • ceramidase deficiency — cer·am·i·dase de·fi·cien·cy (sə ramґĭ dās) Farber disease …   Medical dictionary

  • ASAH1 — N acylsphingosine amidohydrolase (acid ceramidase) 1, also known as ASAH1, is a human gene.cite web | title = Entrez Gene: ASAH1 N acylsphingosine amidohydrolase (acid ceramidase) 1| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene… …   Wikipedia

  • ASAH2 — N acylsphingosine amidohydrolase (non lysosomal ceramidase) 2, also known as ASAH2, is a human gene.cite web | title = Entrez Gene: ASAH2 N acylsphingosine amidohydrolase (non lysosomal ceramidase) 2| url =… …   Wikipedia

  • Disseminierte Lipogranulomatose — Klassifikation nach ICD 10 E75.2 Sonstige Sphingolipidosen (inkl. Farber Krankheit) …   Deutsch Wikipedia

  • Farber-Krankheit — Klassifikation nach ICD 10 E75.2 Sonstige Sphingolipidosen (inkl. Farber Krankheit) …   Deutsch Wikipedia

  • Farber-Syndrom — Klassifikation nach ICD 10 E75.2 Sonstige Sphingolipidosen (inkl. Farber Krankheit) …   Deutsch Wikipedia

  • Morbus Farber — Klassifikation nach ICD 10 E75.2 Sonstige Sphingolipidosen (inkl. Farber Krankheit) …   Deutsch Wikipedia

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