生物来源
goat
质量水平
偶联物
unconjugated
抗体形式
affinity isolated antibody
抗体产品类型
primary antibodies
克隆
polyclonal
形式
buffered aqueous solution
种属反应性
human, canine, mouse, rat
技术
indirect ELISA: suitable
western blot: suitable
UniProt登记号
运输
dry ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... PAFAH1B1(5048)
一般描述
PAFAH1B1 (Platelet activating factor acetyl hydrolase 1b regulatory subunit 1) encodes a protein referred to as Lis1. It is located on the chromosome location 17p13.3. The gene spans ~92 kb at the genomic level. It consists of a N-terminal coiled-coil domain and seven WD40 repeats at the C-terminus end.
免疫原
Peptide with sequence TGSVDQTVKVWECR from the C Terminus of the protein sequence according to NP_000421.
应用
Anti-LIS1/PAFAH1B1 antibody produced in goat is suitable for indirect ELISA and western blot applications.
生化/生理作用
PAFAH1B1 (Platelet activating factor acetyl hydrolase 1b regulatory subunit 1) majorly participates in the neuronal migration pathway during brain development. It is a non-catalytic regulatory subunit of platelet activating factor (PAF) acetyl hydrolase 1b (Pafah1b) complex. During neuronal migration, it conjugates with PAFAH1B2 and PAFAH1B3 to control the concentration of platelet activating factor in the brain. Deleted gene expression of PAFAH1B1 has been reported in the 7p13.3 deletion syndrome or Miller-Dieker syndrome with mental retardation and facial dysmorphism. Heterozygous mutations of the gene cause type 1 lissencephaly characterized with impaired neuronal migration and reduction in the number of cortical gyri.
特点和优势
Evaluate our antibodies with complete peace of mind. If the antibody does not perform in your application, we will issue a full credit or replacement antibody. Learn more.
外形
Supplied at 0.5 mg/mL in Tris saline with 0.02% sodium azide and 0.5% bovine serum albumin.
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警示用语：
Warning
危险声明
预防措施声明
危险分类
Eye Irrit. 2 - Skin Irrit. 2
WGK
WGK 2
闪点(°F)
Not applicable
闪点(°C)
Not applicable
法规信息
新产品
Meng-Han Tsai et al.
Acta neuropathologica, 147(1), 13-13 (2024-01-09)
The development of the cerebral cortex involves a series of dynamic events, including cell proliferation and migration, which rely on the motor protein dynein and its regulators NDE1 and NDEL1. While the loss of function in NDE1 leads to microcephaly-related
Manuel Schiff et al.
European journal of medical genetics, 53(5), 303-308 (2010-07-06)
The 17p13.3 deletion syndrome (or Miller-Dieker syndrome, MDS, MIM 247200) is characterized by lissencephaly, mental retardation and facial dysmorphism. The phenotype is attributed to haploinsufficiency of two genes present in the minimal critical region of MDS: PAFAH1B1 (formerly referred to
Andrew Kodani et al.
Neuron, 106(2), 246-255 (2020-02-26)
Genes mutated in human neuronal migration disorders encode tubulin proteins and a variety of tubulin-binding and -regulating proteins, but it is very poorly understood how these proteins function together to coordinate migration. Additionally, the way in which regional differences in
Amir H Assadi et al.
Neuroscience letters, 439(1), 100-105 (2008-06-03)
Reelin, an extracellular protein that signals through the Dab1 adapter protein, and Lis1 regulate neuronal migration and cellular layer formation in the brain. Loss of Reelin and reduction in Lis1 activity in mice or humans results in the disorganization of
Carlos Cardoso et al.
Human mutation, 19(1), 4-15 (2001-12-26)
Classical lissencephaly (LIS) and subcortical band heterotopia (SBH) are related cortical malformations secondary to abnormal migration of neurons during early brain development. Approximately 60% of patients with classical LIS, and one patient with atypical SBH have been found to have
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