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anti-SMPD1 antibody :: Rabbit anti-Human Acid Sphingomyelinase Polyclonal Antibody

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Catalog # MBS395889
Unit / Price
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  0.1 mg  /  $460 +1 FREE 8GB USB
Western Blot/Blotting
Product Name

Acid Sphingomyelinase (SMPD1), Polyclonal Antibody

Popular Item
Full Product Name

Acid Sphingomyelinase

Product Synonym Names
Acid Sphingomyelinase; aSMase; SMPD1; ASM; Sphingomyelin phosphodiesterase; ASM-1
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 11; NC_000011.9 (6411644..6416228). Location: 11p15.4-p15.1
OMIM
257200
3D Structure
ModBase 3D Structure for P17405
Clonality
Polyclonal
Host
Rabbit
Species Reactivity
Human
Purity/Purification
Ammonium Sulfate Precipitation
Form/Format
Provided as solution in phosphate buffered saline with 0.08% sodium azide
Immunogen
Synthetic peptide derived from human acid sphingomyelinase protein.
Preparation and Storage
Product should be stored at -20 degree C. Aliquot to avoid freeze/thaw cycles
Other Notes
Small volumes of anti-SMPD1 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-SMPD1 antibody
Human acid sphingomyelinase (sphingomyelin phosphodiesterase, ASM) is the lysosomal enzyme responsible for the hydrolysis of sphingomyelin to ceramide and phosphocholine. Converts sphingomyelin to ceramide. aSM also has phospholipase C activities toward 1,2-diacylglycerol-phosphocholine and 1,2-diacylglycerol-phosphoglycerol. The enzyme is a membrane-associated glycoprotein with a pH optimum of about 4.5 and a subunit molecular mass of about 72 kDa. In addition AtoS M, two other sphingomyelinases have been identified in man, a Mg2+- dependent neutral sphingomyelinase found primarily in brain and a Zn2+-dependent acid sphingomyelinase found primarily in serum. Although it is likely that the acid and neutral sphingomyelinases are coded by different genes, the molecular genetic relationship of these three biochemically distinct sphingomyelinases has not been determined. Understanding the role of these sphingomyelinases in the hydrolysis of sphingomyelin to ceramide will be an important step in the understanding of ceramide as it is further hydrolyzed to sphingosine, a neutral phospholipid which has been implicated in the regulation of protein kinase C-mediated signal transduction. Inherited deficiencies of ASM have been reported in man, deficient ASM activity results in the two major subtypes of Niemann-Pick disease (NPD).
Product Categories/Family for anti-SMPD1 antibody
Applications Tested/Suitable for anti-SMPD1 antibody
Western Blot (WB), ELISA (EIA)

Western Blot/Blotting of anti-SMPD1 antibody
Western blot analysis using acid sphingomyelinase antibody on normal human brain lysate (7 ug/lane). Antibody used at 1 ug/ml (1) and 0.5 ug/ml (2) and detected using mouse anti-rabbit antibody at 1:75k dilution and visualized using Pierce West Femto substrate.
anti-SMPD1 antibody Western Blot/Blotting (WB) image
NCBI/Uniprot data below describe general gene information for SMPD1. 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
NCBI Official Full Name
sphingomyelin phosphodiesterase isoform 1
NCBI Official Synonym Full Names
sphingomyelin phosphodiesterase 1, acid lysosomal
NCBI Official Symbol
SMPD1  [Similar Products]
NCBI Official Synonym Symbols
ASM; NPD; ASMASE
  [Similar Products]
NCBI Protein Information
sphingomyelin phosphodiesterase; acid sphingomyelinase
UniProt Protein Name
Sphingomyelin phosphodiesterase
UniProt Synonym Protein Names
Acid sphingomyelinase
UniProt Gene Name
SMPD1  [Similar Products]
UniProt Synonym Gene Names
ASM; aSMase  [Similar Products]
UniProt Entry Name
ASM_HUMAN
NCBI Summary for SMPD1
The protein encoded by this gene is a lysosomal acid sphingomyelinase that converts sphingomyelin to ceramide. The encoded protein also has phospholipase C activity. Defects in this gene are a cause of Niemann-Pick disease type A (NPA) and Niemann-Pick disease type B (NPB). Multiple transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2010]
UniProt Comments for SMPD1
Function: Converts sphingomyelin to ceramide. Also has phospholipase C activities toward 1,2-diacylglycerolphosphocholine and 1,2-diacylglycerolphosphoglycerol. Isoform 2 and isoform 3 have lost catalytic activity.

Catalytic activity: Sphingomyelin + H2O = N-acylsphingosine + phosphocholine.

Subunit structure: Monomer.

Subcellular location: Lysosome.

Polymorphism: A common polymorphism arises from a variable number of hexanucleotide repeat sequence within the signal peptide region.

Involvement in disease: Niemann-Pick disease A (NPDA) [MIM:257200]: An early-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, mental retardation, digestive disorders, failure to thrive, major hepatosplenomegaly, and severe neurologic symptoms. The severe neurological disorders and pulmonary infections lead to an early death, often around the age of four. Clinical features are variable. A phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.11 Ref.12 Ref.14 Ref.16 Ref.18 Ref.19 Ref.21 Ref.23 Ref.24 Ref.27 Ref.30 Ref.31Niemann-Pick disease B (NPDB) [MIM:607616]: A late-onset lysosomal storage disorder caused by failure to hydrolyze sphingomyelin to ceramide. It results in the accumulation of sphingomyelin and other metabolically related lipids in reticuloendothelial and other cell types throughout the body, leading to cell death. Clinical signs involve only visceral organs. The most constant sign is hepatosplenomegaly which can be associated with pulmonary symptoms. Patients remain free of neurologic manifestations. However, a phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B. In Niemann-Pick disease type B, onset of the first symptoms occurs in early childhood and patients can survive into adulthood.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.13 Ref.15 Ref.16 Ref.17 Ref.20 Ref.22 Ref.23 Ref.25 Ref.26 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32

Miscellaneous: There are two types of sphingomyelinases: ASM (acid), and NSM (neutral).

Sequence similarities: Belongs to the acid sphingomyelinase family.Contains 1 saposin B-type domain.
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.

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