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Monoclonal Anti-PAFAH1B1 antibody produced in mouse

产品编号:3149760
规格:clone 2C12, purified immunoglobulin, buffered aqueous solution
包装规格:100 μG
产品类别:进口试剂
品牌:Sigma-Aldrich
优惠价:立即咨询
产品价格
产品编号包装单位单价(元)国内现货国外库存询价单
3149760100 μG4880
产品别名

Monoclonal Anti-PAFAH1B1 antibody produced in mouse

Anti-MDCR

Anti-LIS1

Anti-platelet-activating factor acetylhydrolase, isoform Ib, alpha subunit 45kDa

Anti-PAFAH

基本信息
MDL number
MFCD01324139
General description【一般描述】
This locus was identified as encoding a gene that when mutated or lost caused the lissencephaly associated with Miller-Dieker lissencephaly syndrome. This gene encodes the non-catalytic alpha subunit of the intracellular Ib isoform of platelet-activating factor acteylhydrolase, a heterotrimeric enzyme that specifically catalyzes the removal of the acetyl group at the SN-2 position of platelet-activating factor (identified as 1-O-alkyl-2-acetyl-sn-glyceryl-3-phosphorylcholine). Two other isoforms of intracellular platelet-activating factor acetylhydrolase exist: one composed of multiple subunits, the other, a single subunit. In addition, a single-subunit isoform of this enzyme is found in serum. (provided by RefSeq)
Immunogen【免疫原】
PAFAH1B1 (NP_000421, 1 a.a. ~ 110 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
MVLSQRQRDELNRAIADYLRSNGYEEAYSVFKKEAELDVNEELDKKYAGLLEKKWTSVIRLQKKVMELESKLNEAKEEFTSGGPLGQKRDPKEWIPRPPEKYALSGHRSP
Biochem/physiol Actions【生化/生理作用】
PAFAH1B1 (Platelet activating factor acetyl hydrolase 1b regulatory subunit 1) majorly participates in the neuronal migration pathway during brain development. It is a non-catalytic regulatory subunit of platelet activating factor (PAF) acetyl hydrolase 1b (Pafah1b) complex. During neuronal migration, it conjugates with PAFAH1B2 and PAFAH1B3 to control the concentration of platelet activating factor in the brain. Deleted gene expression of PAFAH1B1 has been reported in the 7p13.3 deletion syndrome or Miller-Dieker syndrome with mental retardation and facial dysmorphism. Heterozygous mutations of the gene cause type 1 lissencephaly characterized with impaired neuronal migration and reduction in the number of cortical gyri.
Physical form【外形】
Solution in phosphate buffered saline, pH 7.4
Legal Information【法律信息】
GenBank is a registered trademark of United States Department of Health and Human Services
产品性质
Quality Level【质量水平】
100
biological source【生物来源】
mouse
conjugate【偶联物】
unconjugated
antibody form【抗体形式】
purified immunoglobulin
antibody product type
primary antibodies
clone【克隆】
2C12, monoclonal
form【形式】
buffered aqueous solution
species reactivity
human
technique(s)
indirect ELISA: suitable
western blot: 1-5 μg/mL
isotype【同位素/亚型】
IgG2aκ
GenBank® accession no.
NM_000430
UniProt accession no.【UniProt登记号】
P43034
shipped in【运输】
dry ice
storage temp.【储存温度】
−20℃
Gene Information
human ... PAFAH1B1(5048)
安全信息
Storage Class Code【储存分类代码】
12 - Non Combustible Liquids
Personal Protective Equipment【个人防护装备】
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
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