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Details for Product No. ABIN150052

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

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Antigen
Synonyms lis2, mdcr, mds, pafah, pafah1b1-b, LIS-1, LIS1, PAFAHA, Lis1, MMS10-U, Mdsh, Ms10u, Pafaha, MDS, LIS2, MDCR, PAFAH
Reactivity
»Alternatives Chicken
Host
»Alternatives Rabbit
Clonality Polyclonal
Conjugate
»Alternatives Un-conjugated
Application
»Alternatives Immunoprecipitation (IP), Western Blotting (WB)
Pubmed 3 references available
Catalog no. ABIN150052
Quantity 50 µg
Price
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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 Human, Mouse (Murine), Rat (Rattus), Xenopus laevis
Alternative Name PAFAH1B1 / LIS1
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
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.
Restrictions For Research Use only
Concentration 1 mg/ml
Buffer Tris-citrate/phosphate, pH 7-8, 0.1% sodium azide
Preservative Sodium azide
Storage 4 °C
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).

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).

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).

Alternatives for antigen "Platelet-Activating Factor Acetylhydrolase 1b, Regulatory Subunit 1 (45kDa) (PAFAH1B1)", type "Antibodies"
Hosts (41), (9), (6)
Reactivities (50), (30), (30), (6), (6), (6), (4), (3), (2)
Applications (44), (25), (12), (10), (8), (5), (2), (1)
Conjugates (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Epitopes (14), (5), (4), (2), (1), (1)
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