SMPD1 Antikörper (AA 201-300) (Cy7)

Produktnummer ABIN883797

Dieses Anti-SMPD1-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von SMPD1 in WB, IF (cc) und IF (p). Geeignet für Human, Maus und Ratte.

Kurzübersicht für SMPD1 Antikörper (AA 201-300) (Cy7) (ABIN883797)

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

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SMPD1 Antikörper ist konjugiert mit Cy7

Applikation: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Produktdetails anti-SMPD1 Antikörper (Cy7)

Bindungsspezifität: AA 201-300

Kreuzreaktivität: Human, Maus, Ratte

Homologie: Dog,Cow,Pig,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human Acid sphingomyelinase

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu SMPD1

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

Andere Bezeichnung: Acid sphingomyelinase

Hintergrund:

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.

Gen-ID: 6609

UniProt: P17405