Rabbit anti-Human PAFAH1B1 Polyclonal Antibody

Uniprot Id

P43034

Target Species

Human

Target Name

PAFAH1B1

Target Full Name

Platelet-activating factor acetylhydrolase IB subunit beta

Target Function

Regulatory subunit (beta subunit) of the cytosolic type I platelet-activating factor (PAF) acetylhydrolase (PAF-AH (I)), an enzyme that catalyzes the hydrolyze of the acetyl group at the sn-2 position of PAF and its analogs and participates in PAF inactivation. Regulates the PAF-AH (I) activity in a catalytic dimer composition-dependent manner. Required for proper activation of Rho GTPases and actin polymerization at the leading edge of locomoting cerebellar neurons and postmigratory hippocampal neurons in response to calcium influx triggered via NMDA receptors. Positively regulates the activity of the minus-end directed microtubule motor protein dynein. May enhance dynein-mediated microtubule sliding by targeting dynein to the microtubule plus end. Required for several dynein- and microtubule-dependent processes such as the maintenance of Golgi integrity, the peripheral transport of microtubule fragments and the coupling of the nucleus and centrosome. Required during brain development for the proliferation of neuronal precursors and the migration of newly formed neurons from the ventricular/subventricular zone toward the cortical plate. Neuronal migration involves a process called nucleokinesis, whereby migrating cells extend an anterior process into which the nucleus subsequently translocates. During nucleokinesis dynein at the nuclear surface may translocate the nucleus towards the centrosome by exerting force on centrosomal microtubules. May also play a role in other forms of cell locomotion including the migration of fibroblasts during wound healing. Required for dynein recruitment to microtubule plus ends and BICD2-bound cargos. May modulate the Reelin pathway through interaction of the PAF-AH (I) catalytic dimer with VLDLR.

Target Involvement

Lissencephaly 1 (LIS1); Subcortical band heterotopia (SBH); Miller-Dieker lissencephaly syndrome (MDLS)

Target Subcellular Location

Cytoplasm, cytoskeleton. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Cytoplasm, cytoskeleton, spindle. Nucleus membrane.

Target Protein Families

WD repeat LIS1/nudF family

Target Tissue Specificity

Fairly ubiquitous expression in both the frontal and occipital areas of the brain.

Target Synonyms

LIS 1; LIS 2; LIS-1; LIS1; LIS1_HUMAN; LIS2; Lissencephaly 1 protein; Lissencephaly-1 protein; MDCR; MDS; PAF acetylhydrolase 45 kDa subunit; PAF AH 45 kDa subunit; PAF AH alpha; PAF-AH 45 kDa subunit; PAF-AH alpha; PAFAH alpha; PAFAH; PAFAH1B1; PAFAHA; Platelet activating factor acetylhydrolase 1b regulatory subunit 1; Platelet activating factor acetylhydrolase isoform Ib alpha subunit; Platelet-activating factor acetylhydrolase IB subunit alpha

Target Background

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.