ASAH1 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IF, IHC, WB
Recommend Dilution	WB: 1 - 2 ug/mL, ICC: 2.5 ug/mL, IF: 20 ug/mL
Reactivity	Human, Mouse, Rat
Host	Rabbit
Clonality	Polyclonal
Immunogen	ASAH1 antibody was raised against a 16 amino acid synthetic peptide near the carboxy terminus of the human ASAH1. The immunogen is located within amino acids 240 - 290 of ASAH1.
Formulation	PBS containing 0.02% sodium azide.
Purification	Affinity chromatography purified via peptide column
Conjugation	Unconjugated
Storage Condition	Store at -20°C as received.
Gene Name	N-acylsphingosine amidohydrolase (acid ceramidase) 1
Database Link	NP_001120977 Entrez Gene 11886 MouseEntrez Gene 84431 RatEntrez Gene 427 Human UniProt ID Q13510
Background	Sphingolipids are hydrolyzed by ceramidases to yield sphingosine and fatty acids. These ceramidases are classified according to the pH range that supports their optimal activity. ASAH1 is an acid ceramidase and key regulator of ceramide metabolism. Mutations in this gene results in Farber Lipogranulomatosis, a fatal human genetic disorder that results in the painful swelling of the joints and tendons and pulminary insufficiency, while a complete knockout of its expression is lethal in mice. Recent studies have shown elevated levels of ASAH1 in Alzheimer’s disease (AD) patients correlating with a reduction in sphingomyelin and elevation of ceramide. Pretreatment of cultured neurons with recombinant AHAH1 prevented the cells from undergoing A-beta (Ab)-induced apoptosis. Multiple isoforms of this protein are known to exist.
Synonyms	AC; ACDase; ASAH; PHP; PHP32; SMAPME
Reference Data
Protein Families	Druggable Genome
Protein Pathways	Lysosome, Metabolic pathways, Sphingolipid metabolism

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