SMPD1 anticorps (AA 201-300) (Biotin)

N° du produit ABIN883807

Détail du produit anti-SMPD1 anticorps (Biotin)

Antigène: SMPD1 (Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1))

Épitope: AA 201-300

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp SMPD1 est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Réactivité croisée: Humain, Souris, Rat

Homologie: Dog,Cow,Pig,Rabbit

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human Acid sphingomyelinase

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

Date de péremption: 12 months

Détails sur SMPD1

Antigène: SMPD1 (Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1))

Autre désignation: Acid sphingomyelinase (SMPD1 Produits)

Synonymes: anticorps ASM, anticorps ASMASE, anticorps NPD, anticorps A-SMase, anticorps Zn-SMase, anticorps aSMase, anticorps SMPD1, anticorps sphingomyelin phosphodiesterase 1, anticorps sphingomyelin phosphodiesterase 1, acid lysosomal, anticorps sphingomyelin phosphodiesterase, anticorps SMPD1, anticorps Smpd1, anticorps LOC5578088

Sujet:

Synonyms: Acid sphingomyelinase, ASM, ASM_HUMAN, aSMase, NPD, Smpd1, Sphingomyelin phosphodiesterase 1 acid lysosomal, Sphingomyelin phosphodiesterase.

Background: 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.Involvement in disease: Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) , also known as Niemann-Pick disease classical infantile form. It is 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.

ID gène: 6609

UniProt: P17405