货号：AB5413

品牌：Merck Millipore

规格：EA

目录价：询价

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Lissencephaly-1 Antibody | AB5413 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Lissencephaly-1 (LIS1). SPECIES REACTIVITIES: Other species have not been tested. It is expected that the antibody may also react with rat, mouse, porcine, bovine, chicken and Xenopus due to sequence homology. Other species have not been tested. View All» Immunogen: Synthetic peptide corresponding to a portion of human Lissencephaly-1 encoded in part by exon 3 and 4. View All» Species Reactivity: Human View All» Application Notes: Western blot: 1:500-1:5,000 Optimal working dilutions must be determined by the end user. View All» Presentation: Affinity purified immunoglobulin. Liquid in Tris-citrate/phosphate, pH 7-8 containing 0.1% sodium azide. View All» Storage Conditions: Maintain at 2-8°C in undiluted for up to 6 months after date of receipt. View All» UniProt Number: P43034 View All» Entrez Gene Number: NM_000430.2 View All» Gene Symbol:

• PAFAH1B1

• LIS2

• PAFAH

• MDS

• LIS-1

• LIS1

• PAFAHA

• MDCR
  View All» Alternate Names: LIS1 View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications: Western Blotting View All» Entrez Gene Summary: PAFAH1B1 was identified as encoding a gene that when mutated or lost caused the lissencephaly associated with Miller-Dieker lissencephaly syndrome. PAFAH1B1 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. View All» UniProt Summary: FUNCTION: SwissProt: P43034 # 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. Non-catalytic subunit of an acetylhydrolase complex which inactivates platelet- activating factor (PAF) by removing the acetyl group at the SN-2 position (By similarity). 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.SIZE: 410 amino acids; 46638 Da SUBUNIT: Component of cytosolic PAF-AH IB, which is composed of PAFAH1B1 (alpha), PAFAH1B2 (beta) and PAFAH1B3 (gamma) subunits. Trimer formation is not essential for the catalytic activity of the enzyme which is contributed solely by the PAFAH1B2 (beta) and PAFAH1B3 (gamma) subunits. Interacts with IQGAP1, KATNB1 and NUDC. Interacts with DAB1 when DAB1 is phosphorylated in response to RELN/reelin signaling (By similarity). Can self-associate. Interacts with DCX, dynein, dynactin, NDE1, NDEL1 and RSN. Interacts with DISC1, and this interaction is enhanced by NDEL1.SUBCELLULAR LOCATION: Cytoplasm, cytoskeleton. Spindle (By similarity). Nucleus membrane (Potential). Centrosome. Note=Redistributes to axons during neuronal development. Also localizes to the microtubules of the manchette in elongating spermatids and to the meiotic spindle in spermatocytes (By similarity). Localizes to the plus end of microtubules and to the centrosome. May localize to the nuclear membrane.TISSUE SPECIFICITY: Fairly ubiquitous expression in both the frontal and occipital areas of the brain.DOMAIN: SwissProt: P43034 Dimerization mediated by the LisH domain may be required to activate dynein (By similarity).DISEASE: SwissProt: P43034 # Defects in PAFAH1B1 are the cause of classical lissencephaly-1 (LIS1) [MIM:607432]. LIS1 is a brain malformation caused by abnormal neuronal migration at 9 to 13 weeks' gestation. Lissencephaly means 'smooth brain', a brain without convolutions or gyri, and is characterized by an absence (agyria) or a decrease (pachygyria) of surface convolutions associated with a disorganization of the clear neuronal lamination of normal six- layered cortex. It is also associated with 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. & Defects in PAFAH1B1 are the cause of subcortical band heterotopia (SBH) [MIM:607432]. SBH is at the less severe end of the lissencephaly spectrum of malformations, characterized by bilateral and symmetric ribbons of gray matter found in the central white matter between the cortex and the ventricular surface. & Defects in PAFAH1B1 are a cause of Miller-Dieker lissencephaly syndrome (MDLS) [MIM:247200]. MDLS is a form of lissencephaly associated with facial abnormalities.SIMILARITY: Belongs to the WD repeat LIS1/nudF family. & Contains 1 LisH domain. & Contains 7 WD repeats. View All» Brand Family: Chemicon View All» Product Name: Anti-Lissencephaly-1 Antibody | AB5413 View All» Concentration: 1 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 50 µg View All» Format: Affinity Purified View All» Host: Rabbit View All»
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