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Farber disease (also
known as Farber's lipogranulomatosis, acid
ceramidase deficiency, "Lipogranulomatosis", and ASAH1-related disorders) is an extremely...
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Ceramidase (EC 3.5.1.23,
acylsphingosine deacylase,
glycosphingolipid ceramide deacylase) is an
enzyme which cleaves fatty acids from ceramide, producing...
- the
generation of ceramides.
Ceramide can be
further hydrolyzed by acid
ceramidase to form
sphingosine and a free
fatty acid, both of
which are able to leave...
- The ASAH1 gene
encodes in
humans the acid
ceramidase enzyme. This gene
encodes a
heterodimeric protein consisting of a
nonglycosylated alpha subunit and...
- Rivera. The ASAH1 gene
codes for acid
ceramidase, an
enzyme found in lysosomes. The
lysosome breaks down acid
ceramidase; the
fatty acid
component is then...
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Alkaline ceramidase 1 also
known as ACER1 is a
ceramidase enzyme which in
humans is
encoded by the ACER1 gene. ACER1
mediates cellular differentiation...
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causing buildup.
Lysosomal storage diseases include:
Sphingolipidoses Ceramidase Farber disease Krabbe disease Infantile onset Late
onset Galactosialidosis...
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erythrocytic Arc/Arg3.1 ASAH1: N-acylsphingosine
amidohydrolase (acid
ceramidase) 1 ASPH:
encoding enzyme Aspartyl/asparaginyl beta-hydroxylase AZIN1:...
-
generated by this process. Finally,
ceramide may be
broken down by a
ceramidase to form sphingosine.
Sphingosine may be
phosphorylated to form sphingosine-1-phosphate...
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Neutral ceramidase is an
enzyme that in
humans is
encoded by the ASAH2 gene. GRCh38:
Ensembl release 89: ENSG00000188611 – Ensembl, May 2017 GRCm38: Ensembl...
