| Long name: |
Acid sphingomyelinase Antibody |
| Also known as: |
Acid sphingomyelinase Antibody |
| Category: |
Primary Antibodies |
| Conjugation: |
Unconjugated |
| Target Antigen: |
Acid sphingomyelinase |
| Specificity: |
This is a highly specific antibody against Acid sphingomyelinase |
| Modification(s): |
None |
| Modification site(s): |
Unmodified antibody |
| Clonality: |
Polyclonal antibody |
| Clone number: |
Polyclonal antibody |
| Concentration: |
1ug per 1ul |
| Source: |
This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human Acid sphingomyelinase |
| Gene ID number: |
6609 |
| Tested Applications: |
IF(IHC-P), IHC-P, WB |
| Recommended dilutions: |
IF(IHC-P)(1:50-200), IHC-P(1:100-500), WB(1:100-1000) |
| Cross reactivity: |
Mouse, Rat, Human |
| Cross reactive species details: |
not every possible cross-reactivity is known, Due to limited amount of testing and knowledge |
| Background of the target antigen: |
A phenotypic continuum exists between type A (basic neurovisceral) and type B (purely visceral) forms of Niemann-Pick disease, Also has phospholipase C activities toward 1, Clinical features are variable, Isoform 2 and isoform 3 have lost catalytic activity, 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, Niemann-Pick disease type A is a primarily neurodegenerative disorder characterized by onset within the first year of life, The severe neurological disorders and pulmonary infections lead to an early death, also known as Niemann-Pick disease classical infantile form, and severe neurologic symptoms, and the intermediate types encompass a cluster of variants combining clinical features of both types A and B, digestive disorders, failure to thrive, leading to cell death, major hepatosplenomegaly, mental retardation, often around the age of four, 2-diacylglycerolphosphocholine and 1, 2-diacylglycerolphosphoglycerol, Converts sphingomyelin to ceramide, Involvement in disease: Defects in SMPD1 are the cause of Niemann-Pick disease type A (NPDA) |
| Purification method: |
This antibody was purified via Protein A |
| Storage conditions: |
50% glycerol and 0, Store at -20°, 09% sodium azide, C for up to 1 year, Keep the antibody in aqueous buffered solution containing 1% BSA |
| Synonym names: |
ASM, ASM_HUMAN, NPD, Smpd1, Sphingomyelin phosphodiesterase, Sphingomyelin phosphodiesterase 1 acid lysosomal, aSMase, Acid sphingomyelinase |
| Properties: |
C, C for long term storage and for short term at + 5°, If you buy Antibodies supplied by Bioss Primary Unconjugated Antibodies they should be stored frozen at - 24° |
| Gene target: |
Acid sphingomyelinase |
| Short name: |
Acid sphingomyelinase Antibody |
| Technique: |
antibodies against human proteins, antibodies for, Antibody |
| Alternative name: |
Acid sphingomyelinase (Antibody to) |
| Alternative technique: |
antibodies |
| Identity: |
11120 |
| Gene: |
SMPD1 |
More about : SMPD1 |
| Long gene name: |
sphingomyelin phosphodiesterase 1 |
| Synonyms gene name: |
acid lysosomal , sphingomyelin phosphodiesterase 1 |
| Synonyms: |
ASM |
| Synonyms name: |
acid sphingomyelinase |
| Locus: |
11p15, 4 |
| Discovery year: |
1986-01-01 |
| GenBank acession: |
AB209775 |
| Entrez gene record: |
6609 |
| Pubmed identfication: |
1711683 |
| RefSeq identity: |
NM_000543 |
| Havana BLAST/BLAT: |
OTTHUMG00000165453 |