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Platelet-Activating Factor Acetylhydrolase 1b, Regulatory Subunit 1 (45kDa) (PAFAH1B1) (Regulatory Subunit 1) antibody

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Synonyms LIS1, LIS2, MDCR, MDS, PAFAH, lis2, mdcr, mds, pafah, pafah1b1-b, LIS-1, PAFAHA, Lis1, MMS10-U, Mdsh, Ms10u, Pafaha
Regulatory Subunit 1
(24), (6), (3), (3), (3), (2), (1), (1), (1), (1), (1)
(65), (35), (33), (7), (7), (6), (2), (2), (2), (1), (1)
(39), (20), (7)
(2), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Immunoprecipitation (IP), Western Blotting (WB)
(51), (33), (12), (11), (5), (3), (3), (2), (2), (1), (1)
Pubmed 3 references available
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Quantity 50 μg
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Immunogen Synthetic peptide (Human) - which represents a portion of human Lissencephaly 1 (Lis 1) protein encoded inpart by exons 3 and 4.
Isotype IgG
Cross-Reactivity Mouse (Murine), Cow (Bovine), Pig (Porcine), Rat (Rattus), Xenopus laevis
Purification Affinity purified using the immunising peptide immobilized on solid support..
Alternative Name PAFAH1B1 / LIS1 (PAFAH1B1 Antibody Abstract)
Background Lissencephaly (LIS), literally meaning smooth brain, has multiple causes. Agyria, i.e., brain without convolutions or gyri, was considered a rare malformation until recent progress in neuroradiology (1). With this technical advantage, a number of lissencephaly syndromes have been distinguished. Classic lissencephaly (type I) is a brain malformation caused by abnormal neuronal migration at 9 to 13 weeks' gestation, resulting in a spectrum of agyria, mixed agyria / pachygyria, and pachygyria. It is characterized by an abnormally thick and poorly organized cortex with 4 primitive layers, diffuse neuronal heterotopia, enlarged and dysmorphic ventricles, and often hypoplasia of the corpus callosum. Lissencephaly is found in association with facial abnormalities in Miller-Dieker syndrome and without other major anomalies in X-linked lissencephaly and isolated lissencephaly sequence (ILS) (2). Subcortical band heterotopia (SBH) are bilateral and symmetric ribbons of gray matter found in the central white matter between the cortex and the ventricular surface and comprise the less severe end of the lissencephaly spectrum of malformations (3).
Synonyms: 5048, PAFAH, LIS1, MDCR, 601545, PAFAH1B1, P43034
Application Notes IP: Use at a concentration of 2-10 ul. WB: Use at a dilution of 1/300-1/3000. Not tested in other applications. Optimal dilutions/concentrations should be determined by the end user.

Positive Controls: TF-1 (erythroleukemia cell line) cell lysate

Restrictions For Research Use only
Format Liquid
Concentration 1 mg/mL
Buffer Tris-citrate/phosphate, pH 7-8, 0.1% sodium azide
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice Do not freeze
Storage 4 °C
Storage Comment Keep as concentrated solution, aliquot and store at 4°C.
Supplier Images
Western Blotting (WB) image for anti-Platelet-Activating Factor Acetylhydrolase 1b, Regulatory Subunit 1 (45kDa) (PAFAH1B1) (Regulatory Subunit 1) antibody (ABIN150052) anti-Platelet-Activating Factor Acetylhydrolase 1b, Regulatory Subunit 1 (45kDa) (PAFAH1B1) (Regulatory Subunit 1) antibody
Background publications Pilz, Kuc, Matsumoto et al.: "Subcortical band heterotopia in rare affected males can be caused by missense mutations in DCX (XLIS) or LIS1." in: Human molecular genetics, Vol. 8, Issue 9, pp. 1757-60, 2000 (PubMed).

Lo Nigro, Chong, Smith et al.: "Point mutations and an intragenic deletion in LIS1, the lissencephaly causative gene in isolated lissencephaly sequence and Miller-Dieker syndrome." in: Human molecular genetics, Vol. 6, Issue 2, pp. 157-64, 1997 (PubMed).

Bordarier, Robain, Rethore et al.: "Inverted neurons in agyria. A Golgi study of a case with abnormal chromosome 17." in: Human genetics, Vol. 73, Issue 4, pp. 374-8, 1986 (PubMed).

Catalog No. ABIN150052
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