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Rabbit Anti-KBP/BF594 Conjugated antibody (bs-9716R-BF594)
訂購熱線:400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-9716R-BF594
英文名稱 Rabbit Anti-KBP/BF594 Conjugated antibody
中文名稱 BF594標記的KBP蛋白抗體
別    名 Hypothetical protein LOC26128; KBP; KBP_HUMAN; KIAA1279; KIF1-binding protein; TTC20; Uncharacterized protein KIAA1279.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  信號轉導  細胞周期蛋白  細胞分化  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,  (predicted: Mouse, Rat, Horse, )
產品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 72kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human KBP
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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.

Function:
Required for organization of axonal microtubules, andaxonal outgrowth and maintenance during peripheral and centralnervous system development. Regulates mitochondrial transport bymodulating KIF1B motor activity.

Subunit:
Interacts with KIF1B.

Subcellular Location:
Mitochondrion.

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.

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.

Similarity:
Belongs to the KIF1-binding protein family.

Database links:

Entrez Gene: 100063227 Horse

Entrez Gene: 26128 Human

Entrez Gene: 72320 Mouse

Entrez Gene: 606294 Rat

Omim: 609367 Human

SwissProt: Q96EK5 Human

SwissProt: Q6ZPU9 Mouse

SwissProt: Q4G074 Rat

Unigene: 279580 Human

Unigene: 258955 Mouse

Unigene: 100975 Rat



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