KBP Rabbit pAb
KBP Rabbit pAb
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Application
| WB |
|---|---|
| Primary Accession | Q96EK5 |
| Reactivity | Human |
| Predicted | Mouse, Rat, Horse |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 71814 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human KBP |
| Epitope Specificity | 151-250/621 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Mitochondrion. |
| SIMILARITY | Belongs to the KIF1-binding protein family. |
| SUBUNIT | Interacts with KIF1B. |
| DISEASE | Defects in KIAA1279 are the cause of Goldberg-Shprintzenmegacolon syndrome (GOSHS) [MIM:609460]. GOSHS is characterized bymicrocephaly, mental retardation and facial dysmorphism, as well asphenotypes related to Hirschsprung disease syndrome. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | Chromosome 10 contains over 800 genes and 135 million nucleotides, making up nearly 4.5% of the human genome. PTEN is an important tumor suppressor gene located on chromosome 10 and, when defective, causes a genetic predisposition to cancer development known as Cowden syndrome. The chromosome 10 encoded gene ERCC6 is important for DNA repair and is linked to Cockayne syndrome which is characterized by extreme photosensitivity and premature aging. Tetrahydrobiopterin deficiency and a number of syndromes involving defective skull and facial bone fusion are also linked to chromosome 10. As with most trisomies, trisomy 10 is rare and is deleterious. The KIAA1279 gene product has been provisionally designated KIAA1279 pending further characterization. |
| Gene ID | 26128 |
|---|---|
| Other Names | KIF-binding protein, KIF1-binding protein, Kinesin family binding protein {ECO:0000312|HGNC:HGNC:23419}, KIFBP (HGNC:23419) |
| Target/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. |
| Dilution | WB=1:500-2000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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. |
| Name | KIFBP (HGNC:23419) |
|---|---|
| Function | Activator of KIF1B plus-end-directed microtubule motor activity (PubMed:16225668). Required for organization of axonal microtubules, and axonal outgrowth and maintenance during peripheral and central nervous system development. |
| Cellular Location | Cytoplasm, cytoskeleton |
| Tissue Location | 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 |
For Research Use Only. Not For Use In Diagnostic Procedures.
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
Chromosome 10 contains over 800 genes and 135 million nucleotides, making up nearly 4.5% of the human genome. PTEN is an important tumor suppressor gene located on chromosome 10 and, when defective, causes a genetic predisposition to cancer development known as Cowden syndrome. The chromosome 10 encoded gene ERCC6 is important for DNA repair and is linked to Cockayne syndrome which is characterized by extreme photosensitivity and premature aging. Tetrahydrobiopterin deficiency and a number of syndromes involving defective skull and facial bone fusion are also linked to chromosome 10. As with most trisomies, trisomy 10 is rare and is deleterious. The KIAA1279 gene product has been provisionally designated KIAA1279 pending further characterization.
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