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

Scan QR to view Datasheet Catalog #    MBS395889
Western Blot/Blotting
Unit / Price
0.1 mg  /  $455 +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   

Acid Sphingomyelinase (SMPD1), Polyclonal Antibody

★Popular Item★
 Also Known As   

Acid Sphingomyelinase

 Product Synonym Names    Acid Sphingomyelinase; aSMase; SMPD1; ASM; Sphingomyelin phosphodiesterase; ASM-1
 Product Gene Name   

anti-SMPD1 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 11; NC_000011.9 (6411644..6416228). Location: 11p15.4-p15.1
 OMIM    257200
 3D Structure    ModBase 3D Structure for P17405
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 Clonality    Polyclonal
 Host    Rabbit
 Species Reactivity    Human
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 Purity/Purification    Ammonium Sulfate Precipitation
 Form/Format    Provided as solution in phosphate buffered saline with 0.08% sodium azide
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 Immunogen    Synthetic peptide derived from human acid sphingomyelinase protein.
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 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.
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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    Polyclonal Antibody
 Applications Tested/Suitable for anti-SMPD1 antibody   

Western Blot (WB), ELISA (EIA)

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 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
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NCBI/Uniprot data below describe general gene information for SMPD1. It may not necessarily be applicable to this product.
 NCBI GI #    56117840
 NCBI GeneID    6609
 NCBI Accession #    NP_000534.3 [Other Products]
 NCBI GenBank Nucleotide #    NM_000543.4 [Other Products]
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 UniProt Primary Accession #    P17405 [Other Products]
 UniProt Secondary Accession #    P17406; Q13811; Q16837; Q16841; A8K8M3; E9PKS3 [Other Products]
 UniProt Related Accession #    P17405 [Other Products]
 Molecular Weight    69851 [Similar Products]
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 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
 Protein Family    Acid sphingomyelinase-like phosphodiesterase
 UniProt Gene Name    SMPD1 [Similar Products]
 UniProt Synonym Gene Names    ASM; aSMase [Similar Products]
 UniProt Entry Name    ASM_HUMAN
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 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]
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 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.
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 Research Articles on SMPD1    1. The SMPD1 p.L302P mutation is a novel risk factor for Parkinson disease
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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-SMPD1 antibodyPathways associated with anti-SMPD1 antibody
 Reference Product  PubMed Publications
 GBA antibody  >1 publications with SMPD1 and GBA
 SMPD2 antibody  >1 publications with SMPD1 and SMPD2
 Products by Pathway  Pathway Diagram
 Ceramide Signaling Pathway antibodies  Ceramide Signaling Pathway Diagram
 FAS (CD95) Signaling Pathway antibodies  FAS (CD95) Signaling Pathway Diagram
 Glycosphingolipid Metabolism Pathway antibodies  Glycosphingolipid Metabolism Pathway Diagram
 IL2 Signaling Events Mediated By PI3K Pathway antibodies  IL2 Signaling Events Mediated By PI3K Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Lysosome Pathway antibodies  Lysosome Pathway Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Lipids And Lipoproteins Pathway antibodies  Metabolism Of Lipids And Lipoproteins Pathway Diagram
 Ovarian Infertility Genes Pathway antibodies  Ovarian Infertility Genes Pathway Diagram
 Sphingolipid Metabolism Pathway antibodies  Sphingolipid Metabolism Pathway Diagram
Diseases associated with anti-SMPD1 antibodyOrgans/Tissues associated with anti-SMPD1 antibody
 Disease Name  Pubmed Publications
 Niemann-Pick Diseases Antibodies  >28 publications with SMPD1 and Niemann-Pick Diseases
 Niemann-Pick Disease, Type B Antibodies  >9 publications with SMPD1 and Niemann-Pick Disease, Type B
 Neoplasms Antibodies  >4 publications with SMPD1 and Neoplasms
 Inflammation Antibodies  >4 publications with SMPD1 and Inflammation
 Disease Models, Animal Antibodies  >3 publications with SMPD1 and Disease Models, Animal
 Niemann-Pick Disease, Type A Antibodies  >3 publications with SMPD1 and Niemann-Pick Disease, Type A
 Necrosis Antibodies  >2 publications with SMPD1 and Necrosis
 Cardiovascular Diseases Antibodies  >1 publications with SMPD1 and Cardiovascular Diseases
 Liver Diseases Antibodies  >1 publications with SMPD1 and Liver Diseases
 Hepatomegaly Antibodies  >1 publications with SMPD1 and Hepatomegaly
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >10 publications with SMPD1 and Blood
 Brain Antibodies  >9 publications with SMPD1 and Brain
 Liver Antibodies  >6 publications with SMPD1 and Liver
 Skin Antibodies  >5 publications with SMPD1 and Skin
 Bone Antibodies  >5 publications with SMPD1 and Bone
 Vascular Antibodies  >3 publications with SMPD1 and Vascular
 Lung Antibodies  >3 publications with SMPD1 and Lung
 Adipose Tissue Antibodies  >2 publications with SMPD1 and Adipose Tissue
 Nerve Antibodies  >2 publications with SMPD1 and Nerve
 Connective Tissue Antibodies  >2 publications with SMPD1 and Connective Tissue
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