产品名称
KBP Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/26128
Human Swissprot No.
Q96EK5
Human Swissprot Link
https://www.uniprot.org/uniprotkb/Q96EK5/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/72320
Mouse Swissprot No.
Q6ZPU9
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q6ZPU9
Rat Gene Link
https://www.uniprot.org/uniprot/606294
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/Q4G074
免疫原
Synthesized peptide derived from human KBP AA range: 542-592
特异性
This antibody detects endogenous levels of KBP at Human/Mouse/Rat
稀释度
WB 1:500-2000;IHC-p 1:50-300
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a kinesin family member 1 binding protein that is characterized by two tetratrico peptide repeats. The encoded protein localizes to the mitochondria and may be involved in regulating transport of the mitochondria. Mutations in this gene are associated with Goldberg-Shprintzen megacolon syndrome. [provided by RefSeq, Mar 2010],
组织表达
Highly expressed in heart, brain, ovary, testis, spinal cord and all specific brain regions examined. Moderate expressed at intermediate level in all other adult tissues examined, as well as in fetal liver and brain. Not expressed in blood leukocytes.
细胞定位
Cytoplasm, cytoskeleton .
功能
disease:Defects in KIAA1279 are the cause of Goldberg-Shprintzen megacolon syndrome (GOSHS) [MIM:609460]. GOSHS is characterized by microcephaly, mental retardation and facial dysmorphism, as well as phenotypes related to Hirschsprung disease syndrome.,function:Required for organization of axonal microtubules, and axonal outgrowth and maintenance during peripheral and central nervous system development. Regulates mitochondrial transport by modulating KIF1B motor activity.,similarity:Belongs to the KIF1-binding protein family.,subunit:Interacts with KIF1B.,tissue specificity:Highly expressed in heart, brain, ovary, testis, spinal cord and all specific brain regions examined. Moderate expressed at intermediate level in all other adult tissues examined, as well as in fetal liver and brain. Not expressed in blood leukocytes.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
