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Upingbio
SKU:YP-Ab-11739-100UL
KBP rabbit pAb
KBP rabbit pAb
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- Gene Name: KIAA1279 KBP
- Immunogen: Synthesized peptide derived from human KBP AA range: 542-592
- Specificity: This antibody detects endogenous levels of KBP at Human/Mouse/Rat
- Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- Source: Polyclonal, Rabbit,IgG
- Dilution ratio: WB 1:500-2000;IHC-p 1:50-300
- Purification process: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
- Concentration: 1 mg/ml
- Storage: -20°C/1 year
- Background: 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],
- Function: 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.,
- Species: Human; Mouse;Rat
- Range: WB;IHC
- Protein: KBP
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