Monoclonal Anti-PAFAH1B1 antibody produced in mouse
SIGMA/WH0005048M1 - clone 2C12, purified immunoglobulin, buffered aqueous solution
Synonym: Anti-LIS1; Anti-MDCR; Anti-PAFAH; Anti-
MDL Number: MFCD01324139
Product Type: Chemical
Western Blotting QC Western Blot detection against Immunogen (37.84 kDa).
ELISA Detection limit for recombinant GST tagged PAFAH1B1 is approximately 0.03 ng/mL as a capture antibody.
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| biological source | mouse |
| clone | 2C12, monoclonal |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| GenBank® accession no. | NM_000430   |
| isotype | IgG2aκ |
| Quality Level | 100 |
| shipped in | dry ice |
| species reactivity | human |
| storage temp. | −20°C |
| technique(s) | indirect ELISA: suitable |
| western blot: 1-5 μg/mL | |
| UniProt accession no. | P43034 |
| Biochem/physiol Actions: | 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. |
| General description: | 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-gly |
| Immunogen: | 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 MVLSQRQRDELNRAIADYLRSNGYE |
| Legal Information: | GenBank is a registered trademark of United States Department of Health and Human Services |
| Physical form: | Solution in phosphate buffered saline, pH 7.4 |
| RIDADR | NONH for all modes of transport |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |