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anti-ASAH1 antibody :: Rabbit ASAH1 Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS2519701
Immunohistochemistry (IHC)
Unit / Price
0.02 mL  /  $110 +1 FREE 8GB USB
0.06 mL  /  $150 +1 FREE 8GB USB
0.12 mL  /  $225 +1 FREE 8GB USB
0.2 mL  /  $360 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

ASAH1, Polyclonal Antibody

 Also Known As   

ASAH1 Polyclonal Antibody

 Product Synonym Names    AC; PHP; ASAH; PHP32; ACDase; SMAPME
 Product Gene Name   

anti-ASAH1 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    159950
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Antigen affinity purification
 Concentration    1.6mg/mL (lot specific)
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 Immunogen    Recombinant protein of human ASAH1
 Calculated Molecular Weight: 45kDa   
 Buffer    PBS with 0.05% sodium azide, 50% glycerol, pH7.3
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 Preparation and Storage    Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.
 Other Notes    Small volumes of anti-ASAH1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for anti-ASAH1 antibody

   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.
 Applications Tested/Suitable for anti-ASAH1 antibody   

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

 Application Notes for anti-ASAH1 antibody    WB: 1:500-1:2000
IHC: 1:50-1:200
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 Immunohistochemistry (IHC) of anti-ASAH1 antibody    Immunohistochemistry of Human cervical cancer using ASAH1 Polyclonal Antibody at dilution of 1:40
anti-ASAH1 antibody Immunohistochemistry (IHC) (IHC) image
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NCBI/Uniprot data below describe general gene information for ASAH1. It may not necessarily be applicable to this product.
 NCBI GI #    189011550
 NCBI GeneID    427
 NCBI Accession #    NP_001120977.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001127505.1 [Other Products]
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 UniProt Secondary Accession #    Q6W898; Q96AS2; E9PDS0 [Other Products]
 UniProt Related Accession #    Q13510 [Other Products]
 Molecular Weight    44,046 Da
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 NCBI Official Full Name    acid ceramidase isoform c
 NCBI Official Synonym Full Names    N-acylsphingosine amidohydrolase (acid ceramidase) 1
 NCBI Official Symbol    ASAH1 [Similar Products]
 NCBI Official Synonym Symbols   
AC; PHP; ASAH; PHP32; ACDase; SMAPME
[Similar Products]
 NCBI Protein Information    acid ceramidase; acid CDase; acylsphingosine deacylase; putative 32 kDa heart protein
 UniProt Protein Name    Acid ceramidase
 UniProt Synonym Protein Names   
Acylsphingosine deacylase; N-acylsphingosine amidohydrolase; Putative 32 kDa heart protein; PHP32
 Protein Family    Acid ceramidase
 UniProt Gene Name    ASAH1 [Similar Products]
 UniProt Synonym Gene Names    ASAH; AC; ACDase; Acid CDase; PHP32 [Similar Products]
 UniProt Entry Name    ASAH1_HUMAN
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 NCBI Summary for ASAH1    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, Jul 2008]
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 UniProt Comments for ASAH1    ASAH1: Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid. Defects in ASAH1 are the cause of Farber lipogranulomatosis (FL); also known as Farber disease (FD). This sphingolipid disease is characterized by subcutaneous lipid-loaded nodules, excruciating pain in the joints and extremities, marked accumulation of ceramide in lysosomes, and death by three years of age. Defects in ASAH1 are the cause of spinal muscular atrophy with progressive myoclonic epilepsy (SMAPME). An autosomal recessive neuromuscular disorder characterized by childhood onset of motor deficits and progressive myoclonic seizures, after normal developmental milestones. Proximal muscle weakness and generalized muscular atrophy are due to degeneration of spinal motor neurons. Myoclonic epilepsy is generally resistant to conventional therapy. The disease course is progressive and leads to respiratory muscle involvement and severe handicap or early death from respiratory insufficiency. Belongs to the acid ceramidase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Lipid Metabolism - sphingolipid; Hydrolase; EC 3.5.1.23

Chromosomal Location of Human Ortholog: 8p22

Cellular Component: lysosomal lumen

Molecular Function: catalytic activity; ceramidase activity

Biological Process: response to organic substance; sphingolipid metabolic process; glycosphingolipid metabolic process; ceramide metabolic process; lung development

Disease: Farber Lipogranulomatosis
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 Research Articles on ASAH1    1. Acid ceramidase promotes nuclear export of PTEN through sphingosine 1-phosphate mediated Akt signaling.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-ASAH1 antibodyPathways associated with anti-ASAH1 antibody
 Reference Product  PubMed Publications
 SMPD1 antibody  >62 publications with ASAH1 and SMPD1
 SPHK1 antibody  >3 publications with ASAH1 and SPHK1
 GALC antibody  >2 publications with ASAH1 and GALC
 UGCG antibody  >1 publications with ASAH1 and UGCG
 SPHK2 antibody  >1 publications with ASAH1 and SPHK2
 SGPP1 antibody  >1 publications with ASAH1 and SGPP1
 Products by Pathway  Pathway Diagram
 Ceramide Signaling Pathway antibodies  Ceramide Signaling Pathway Diagram
 Glycosphingolipid Metabolism Pathway antibodies  Glycosphingolipid Metabolism Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Lipids And Lipoproteins Pathway antibodies  Metabolism Of Lipids And Lipoproteins Pathway Diagram
 Signal Transduction Of S1P Receptor Pathway antibodies  Signal Transduction Of S1P Receptor Pathway Diagram
 Sphingolipid Metabolism Pathway antibodies  Sphingolipid Metabolism Pathway Diagram
 Sphingolipid Metabolism Pathway antibodies  Sphingolipid Metabolism Pathway Diagram
Diseases associated with anti-ASAH1 antibodyOrgans/Tissues associated with anti-ASAH1 antibody
 Disease Name  Pubmed Publications
 Lipidoses Antibodies  >29 publications with ASAH1 and Lipidoses
 Farber Lipogranulomatosis Antibodies  >27 publications with ASAH1 and Farber Lipogranulomatosis
 Necrosis Antibodies  >15 publications with ASAH1 and Necrosis
 Liver Diseases Antibodies  >11 publications with ASAH1 and Liver Diseases
 Inflammation Antibodies  >8 publications with ASAH1 and Inflammation
 Fatty Liver Antibodies  >8 publications with ASAH1 and Fatty Liver
 Fibrosis Antibodies  >6 publications with ASAH1 and Fibrosis
 Drug-Induced Liver Injury Antibodies  >4 publications with ASAH1 and Drug-Induced Liver Injury
 Kidney Diseases Antibodies  >4 publications with ASAH1 and Kidney Diseases
 Seizures Antibodies  >4 publications with ASAH1 and Seizures
 Organ/Tissue Name  Pubmed Publications
 Skin Antibodies  >41 publications with ASAH1 and Skin
 Liver Antibodies  >35 publications with ASAH1 and Liver
 Blood Antibodies  >31 publications with ASAH1 and Blood
 Brain Antibodies  >31 publications with ASAH1 and Brain
 Kidney Antibodies  >24 publications with ASAH1 and Kidney
 Lung Antibodies  >22 publications with ASAH1 and Lung
 Prostate Antibodies  >18 publications with ASAH1 and Prostate
 Muscle Antibodies  >18 publications with ASAH1 and Muscle
 Intestine Antibodies  >10 publications with ASAH1 and Intestine
 Vascular Antibodies  >9 publications with ASAH1 and Vascular
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