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anti-ASAH1 antibody :: Rabbit anti-Human ASAH1 Polyclonal Antibody

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Catalog # MBS7052821
Unit / Price
  0.05 mg  /  $160 +1 FREE 8GB USB
  0.1 mg  /  $235 +1 FREE 8GB USB
Product Name

ASAH1, Polyclonal Antibody

Popular Item
Also Known As

ASAH1 Antibody

Product Synonym Names
Acid ceramidase; AC; ACDase; Acid CDase; Acylsphingosine deacylase; N-acylsphingosine amidohydrolase; Putative 32 kDa heart protein; PHP32; Acid ceramidase subunit alpha; Acid ceramidase subunit beta; ASAH1; ASAH; HSD-33; HSD33
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Immunogen Sequence Length
389
OMIM
U70063 mRNA
3D Structure
ModBase 3D Structure for Q13510
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Purity/Purification
>95%, Protein G purified
Form/Format
Liquid
Immunogen
Recombinant Human Acid ceramidase protein (22-395AA)
Conjugation
Non-conjugated
Preservative
0.03% Proclin 300
Constituents
50% Glycerol, 0.01M PBS, pH 7.4
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 9001:2008 Certified Laboratory.
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.
Related Product Information for
anti-ASAH1 antibody
Hydrolyzes the sphingolipid ceramide into sphingosine and free fatty acid.
Applications Tested/Suitable for anti-ASAH1 antibody
ELISA (EIA), Immunohistochemistry (IHC)
Application Notes for anti-ASAH1 antibody
IHC: 1:20-1:200

Immunohistochemistry (IHC) of anti-ASAH1 antibody
Immunohistochemistry of paraffin-embedded human heart tissue using MBS7052821 at dilution of 1:100
anti-ASAH1 antibody Immunohistochemistry (IHC) (IHC) image
Western Blot (WB) of anti-ASAH1 antibody
Western Blot
Positive WB detected in: Hela whole cell lysate
All lanes: ASAH1 antibody at 3ug/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 45,47 KDa
Observed band size: 45 KDa

anti-ASAH1 antibody Western Blot (WB) (WB) image
NCBI/Uniprot data below describe general gene information for ASAH1. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
44,046 Da
NCBI Official Full Name
acid ceramidase isoform c
NCBI Official Synonym Full Names
N-acylsphingosine amidohydrolase 1
NCBI Official Symbol
ASAH1  [Similar Products]
NCBI Official Synonym Symbols
AC; PHP; ASAH; PHP32; ACDase; SMAPME
  [Similar Products]
NCBI Protein Information
acid ceramidase
UniProt Protein Name
Acid ceramidase
UniProt Synonym Protein Names
Acylsphingosine deacylase; N-acylsphingosine amidohydrolase; Putative 32 kDa heart protein; PHP32
Protein Family
UniProt Gene Name
ASAH1  [Similar Products]
UniProt Synonym Gene Names
ASAH; AC; ACDase; Acid CDase; PHP32  [Similar Products]
NCBI Summary for ASAH1
This gene encodes a member of the acid ceramidase family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. Processing of this preproprotein generates alpha and beta subunits that heterodimerize to form the mature lysosomal enzyme, which catalyzes the degradation of ceramide into sphingosine and free fatty acid. This enzyme is overexpressed in multiple human cancers and may play a role in cancer progression. Mutations in this gene are associated with the lysosomal storage disorder, Farber lipogranulomatosis, and a neuromuscular disorder, spinal muscular atrophy with progressive myoclonic epilepsy. [provided by RefSeq, Oct 2015]
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: EC 3.5.1.23; Hydrolase; Lipid Metabolism - sphingolipid

Chromosomal Location of Human Ortholog: 8p22

Cellular Component: extracellular space; lysosomal lumen

Molecular Function: catalytic activity; ceramidase activity

Biological Process: ceramide metabolic process; glycosphingolipid metabolic process

Disease: Farber Lipogranulomatosis
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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