Mouse Anti-Lissencephaly-1 (LIS-1, MDCR, Miller-Dieker Syndrome, MDS, PAF Acetylhydrolase 45kD Subunit, PAFAH 45kD, PAFAH alpha, PAFAH1B1, Platelet Activating Factor Acetylhydrolase Isoform Ib alpha Subunit, PAFAHA, SBH, Subcortical Band Heterotopia, ILS, Isolated Lissencephaly Sequence)
PAFAH alpha 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. 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. It is expressed ubiquitously in both the frontal and occipital areas of the brain. Defects in PAFAH alpha cause lissencephaly type 1 (LIS1) and of subcortical band heterotopia (SBH). Defects in PAFAH alpha are also a cause of Miller-Dieker lissencephaly syndrome (MLDS).
Applications
Suitable for use in Western Blot and ELISA. Other applications have not been tested.
Recommended Dilutions
Optimal dilutions to be determined by the researcher.
Storage and Stability
Lyophilized and reconstituted products are stable for 12 months after receipt at -20°C. Reconstitute with sterile dH2O. Aliquot to avoid repeated freezing and thawing. Store at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Immunogen
Partial recombinant protein corresponding to aa1-111 from human Lissencephaly-1 with a GST tag.
Form
Supplied as a lyophilized powder from PBS, pH 7.2. Reconstitute with 100ul sterile ddH2O.
Purity
Purified by Protein G affinity chromatography.
Specificity
Recognizes human Lissencephaly-1.
