ASAH1 polyclonal antibody
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Specification
Product Description
Rabbit polyclonal antibody raised against synthetic peptide of ASAH1.
Immunogen
A synthetic peptide corresponding to C-terminus 16 amino acids of human ASAH1.
Host
Rabbit
Reactivity
Human, Mouse, Rat
Form
Liquid
Recommend Usage
Western Blot (1 ug/mL)
The optimal working dilution should be determined by the end user.Storage Buffer
In PBS (0.02% sodium azide)
Storage Instruction
Store at 4°C for three months. For long term storage store at -20°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Tissue lysate)
Western blot analysis of ASAH1 in mouse heart tissue lysate with ASAH1 polyclonal antibody (Cat # PAB13056) at (A) 1 and (B) 2 ug/mL .Immunohistochemistry
Immunohistochemical staining of human heart tissue with 2.5 ug/mL ASAH1 polyclonal antibody (Cat # PAB13056). -
Gene Info — ASAH1
Entrez GeneID
427Protein Accession#
EAW63795Gene Name
ASAH1
Gene Alias
AC, ASAH, FLJ21558, FLJ22079, PHP, PHP32
Gene Description
N-acylsphingosine amidohydrolase (acid ceramidase) 1
Omim ID
228000Gene Ontology
HyperlinkGene Summary
This gene encodes a heterodimeric protein consisting of a nonglycosylated alpha subunit and a glycosylated beta subunit that is cleaved to the mature enzyme posttranslationally. The encoded protein catalyzes the synthesis and degradation of ceramide into sphingosine and fatty acid. Mutations in this gene have been associated with a lysosomal storage disorder known as Farber disease. Multiple transcript variants encoding several distinct isoforms have been identified for this gene. [provided by RefSeq
Other Designations
N-acylsphingosine amidohydrolase 1|OTTHUMP00000122482|acylsphingosine deacylase|putative 32 kDa heart protein
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Interactome
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Pathway
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Disease
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Publication Reference
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Deregulation of sphingolipid metabolism in Alzheimer's disease.
He X, Huang Y, Li B, Gong CX, Schuchman EH.
Neurobiology of Aging 2010 Mar; 31(3):398.
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Insertional mutagenesis of the mouse acid ceramidase gene leads to early embryonic lethality in homozygotes and progressive lipid storage disease in heterozygotes.
Li CM, Park JH, Simonaro CM, He X, Gordon RE, Friedman AH, Ehleiter D, Paris F, Manova K, Hepbildikler S, Fuks Z, Sandhoff K, Kolesnick R, Schuchman EH.
Genomics 2002 Feb; 79(2):218.
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Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease.
Koch J, Gartner S, Li CM, Quintern LE, Bernardo K, Levran O, Schnabel D, Desnick RJ, Schuchman EH, Sandhoff K.
The Journal of Biological Chemistry 1996 Dec; 271(51):33110.
Application:IP, Monkey, COS-1 cells.
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Deregulation of sphingolipid metabolism in Alzheimer's disease.
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