WH0005048M1

Monoclonal Anti-PAFAH1B1 antibody produced in mouse

clone 2C12, purified immunoglobulin, buffered aqueous solution

• 동의어(들): Anti-LIS1, Anti-MDCR, Anti-PAFAH, Anti-platelet-activating factor acetylhydrolase, isoform Ib, alpha subunit 45kDa
• MDL number: MFCD01324139
• UNSPSC 코드: 12352203
• NACRES: NA.43

속성

• 생물학적 소스: mouse
• Quality Level: 100
• 결합: unconjugated
• 항체 형태: purified immunoglobulin
• 항체 생산 유형: primary antibodies
• 클론: 2C12, monoclonal
• 양식: buffered aqueous solution
• 종 반응성: human
• 기술: indirect ELISA: suitable; western blot: 1-5 μg/mL
• 동형: IgG2aκ
• GenBank 수납 번호: NM_000430
• UniProt 수납 번호: P43034
• 배송 상태: dry ice
• 저장 온도: −20°C
• 타겟 번역 후 변형: unmodified
• 유전자 정보: human ... PAFAH1B1(5048)

설명

일반 설명

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)

면역원

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

생화학적/생리학적 작용

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.

물리적 형태

Solution in phosphate buffered saline, pH 7.4

법적 정보

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안전성 정보

• Storage Class Code: 10 - Combustible liquids
• Flash Point (°F): Not applicable
• Flash Point (°C): Not applicable
• 개인 보호 장비: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)