SMPD1 Antikörper (AA 201-300) (AbBy Fluor® 488)
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- Target Alle SMPD1 Antikörper anzeigen
- SMPD1 (Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1))
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Bindungsspezifität
- AA 201-300
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Reaktivität
- Human, Maus, Ratte
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Wirt
- Kaninchen
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Klonalität
- Polyklonal
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Konjugat
- Dieser SMPD1 Antikörper ist konjugiert mit AbBy Fluor® 488
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Applikation
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 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
- Top Product
- Discover our top product SMPD1 Primärantikörper
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- Applikationshinweise
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - Beschränkungen
- Nur für Forschungszwecke einsetzbar
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- 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
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- Target
- SMPD1 (Sphingomyelin phosphodiesterase 1, Acid Lysosomal (SMPD1))
- Andere Bezeichnung
- Acid sphingomyelinase (SMPD1 Produkte)
- Synonyme
- ASM antikoerper, ASMASE antikoerper, NPD antikoerper, A-SMase antikoerper, Zn-SMase antikoerper, aSMase antikoerper, SMPD1 antikoerper, sphingomyelin phosphodiesterase 1 antikoerper, sphingomyelin phosphodiesterase 1, acid lysosomal antikoerper, sphingomyelin phosphodiesterase antikoerper, SMPD1 antikoerper, Smpd1 antikoerper, LOC5578088 antikoerper
- Hintergrund
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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
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