CENPJ,着丝粒蛋白J抗体

产品编号HZ-13835R
英文名称CENPJ
中文名称着丝粒蛋白J抗体
别 名CENP-J; CENPJ; CENPJ_HUMAN; Centromere protein J; Centrosomal P4.1-associated protein; CPAP; LAG-3-associated protein; LAP; LIP1; LYST-interacting protein 1; MCPH6.
说 明 书0.1ml 0.2ml
研究领域细胞生物 神经生物学 信号转导 转录调节因子 细胞分化
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat,
CENPJ,着丝粒蛋白J抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量153kDa
细胞定位细胞浆
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human CENPJ
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
CENPJ,着丝粒蛋白J抗体PubMedPubMed
产品介绍background:
This gene encodes a protein that belongs to the centromere protein family. During cell division, this protein plays a structural role in the maintenance of centrosome integrity and normal spindle morphology, and it is involved in microtubule disassembly at the centrosome. This protein can function as a transcriptional coactivator in the Stat5 signaling pathway, and also as a coactivator of NF-kappaB-mediated transcription, likely via its interaction with the coactivator p300/CREB-binding protein. Mutations in this gene are associated with primary autosomal recessive microcephaly, a disorder characterized by severely reduced brain size and mental retardation. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Apr 2012]

Function:
Plays an important role in cell division and centrosome function by participating in centriole duplication. Inhibits microtubule nucleation from the centrosome.

Subcellular Location:
Cytoplasm > cytoskeleton > centrosome. Cytoplasm > cytoskeleton > centrosome > centriole. Localized within the center of microtubule asters. During centriole biogenesis, it is concentrated within the proximal lumen of both parental centrioles and procentrioles.

Post-translational modifications:
Phosphorylation at Ser-589 and Ser-595 by PLK2 is required for procentriole formation and centriole elongation. Phosphorylation by PLK2 oscillates during the cell cycle: it increases at G1/S transition and decreases during the exit from mitosis. Phosphorylation at Ser-595 is also mediated by PLK4 but is not a critical step in PLK4 function in procentriole assembly.

DISEASE:
Defects in CENPJ are the cause of microcephaly primary type 6 (MCPH6) [MIM:608393]. A disorder defined as a head circumference more than 3 standard deviations below the age-related mean. Brain weight is markedly reduced and the cerebral cortex is disproportionately small. Despite this marked reduction in size, the gyral pattern is relatively well preserved, with no major abnormality in cortical architecture. Primary microcephaly is further defined by the absence of other syndromic features or significant neurological deficits.
Defects in CENPJ are the cause of Seckel syndrome type 4 (SCKL4) [MIM:613676].
SCKL4 is a rare autosomal recessive disorder characterized by proportionate dwarfism of prenatal onset associated with low birth weight, growth retardation, severe microcephaly with a bird-headed like appearance, and mental retardation.

Similarity:
Belongs to the TCP10 family.

Gene ID:
55835

CENPJ,着丝粒蛋白J抗体Database links:
Entrez Gene: 55835 Human
Entrez Gene: 219103 Mouse
Omim: 609279 Human
SwissProt: Q9HC77 Human
SwissProt: Q569L8 Mouse
Unigene: 513379 Human
Unigene: 533828 Human
Unigene: 741581 Human
Unigene: 212525 Mouse

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.