Acid sphingomyelinase Antibody, ALEXA FLUOR 647

• Catalog number
  bs-6318R-A647
• Price
  Please ask
• Size
  100ul

• Long name
  Acid sphingomyelinase Antibody, ALEXA FLUOR 647 Conjugated
• Also known as
  Anti-Acid sphingomyelinase PAb ALEXA FLUOR 647
• Category
  Conjugated Primary Antibodies
• Conjugated with
  ALEXA FLUOR® 647
• Host Organism
  Rabbit (Oryctolagus cuniculus)
• Target Antigen
  Acid sphingomyelinase
• Specificity
  This is a highly specific antibody against Acid sphingomyelinase.
• Modification
  Unmodified
• Modification Site
  None
• Clonality
  Polyclonal
• Clone
  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)
• Recommended dilutions
  IF(IHC-P)(1:50-200)
• Crossreactivity
  Human, Mouse, Rat
• Cross reactive species details
  Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
• Background of the antigen
  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.
• Purification
  Purified by Protein A.
• Storage conditions
  Store this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
• Excitation emission
  650nm/665nm
• Synonyms
  Acid sphingomyelinase; ASM; ASM_HUMAN; aSMase; NPD; Smpd1; Sphingomyelin phosphodiesterase 1 acid lysosomal; Sphingomyelin phosphodiesterase.
• Properties
  For facs or microscopy Alexa 1 conjugate. Alexa Fluor 633 is a practical alternative to APC as well as Cy5. Bioss Primary Conjugated Antibodies. ALEXA FLUOR made this Alexa Fluor 633 conjugate that can be used in multi-color flow cytometry with instruments equipped with a second red laser or red diode. It is detected in the FL4 detector of the core's upgraded 2-laser FACScans. Like other Alexa Fluor dyes, the Anti-Acid sphingomyelinase exhibits uncommon photo stability, making it an ideal choice for fluorescent microscopy. If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
• Conjugation
  Alexa Fluor, ALEXA FLUOR 647
• French translation
  anticorps

• Gene target
  Acid   sphingomyelinase  
• Gene symbol
  SMPDL3A, SMPD1
• Short name
  Anti-Acid sphingomyelinase
• Technique
  Antibody, antibodies against human proteins, antibodies for
• Isotype
  Immunoglobulin G (IgG)
• Label
  ALEXA FLUOR 647
• Alternative name
  Acid sphingomyelinase Antibody, ALEXA FLUOR 647
• Alternative technique
  antibodies

Gene info

• Identity
  HGNC:17389
• Gene
  SMPDL3A
• Long gene name
  sphingomyelin phosphodiesterase acid like 3A
• Synonyms gene name
  • sphingomyelin phosphodiesterase acid-like 3A
• Synonyms
  • FLJ20177
  • ASM3A
  • ASML3a
  • yR36GH4.1
• Synonyms name
  • acid sphingomyelinase-like phosphodiesterase 3a
• GenBank acession
  • AK000184
• Locus
  6q22.31
• Discovery year
  2003-11-26
• Entrez gene record
  10924
• Pubmed identfication
  • 12442002
  • 25288789
• RefSeq identity
  • NM_006714
• VEGA ID
  OTTHUMG00000015490

Gene info

• Identity
  HGNC:11120
• Gene
  SMPD1
• Long gene name
  sphingomyelin phosphodiesterase 1
• Synonyms gene name
  • sphingomyelin phosphodiesterase 1, acid lysosomal
• Synonyms
  • ASM
• Synonyms name
  • acid sphingomyelinase
  • Niemann-Pick type A/B
• GenBank acession
  • AB209775
• Locus
  11p15.4
• Discovery year
  1986-01-01
• Entrez gene record
  6609
• Pubmed identfication
  • 1711683
• RefSeq identity
  • NM_000543
• VEGA ID
  OTTHUMG00000165453

MeSH Data

• Name
  Blotting, Western
• Concept
  Scope note: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
• Tree numbers
  • E05.196.401.143
  • E05.301.300.096
  • E05.478.566.320.200
  • E05.601.262
  • E05.601.470.320.200
• Qualifiers
  ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data

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