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Rabbit Anti-HCCS/BF350 Conjugated antibody (bs-15424R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-15424R-BF350
英文名稱 Rabbit Anti-HCCS/BF350 Conjugated antibody
中文名稱 BF350標(biāo)記的全細(xì)胞色素C合成酶抗體
別    名 CCHL; DKFZp779I1858; EC 4.4.1.17; Holocytochrome c synthase (cytochrome c heme lyase); Holocytochrome c type synthase; MCOPS7; OTTHUMP00000022903; OTTHUMP00000022904; OTTHUMP00000022905; OTTMUSP00000021173; OTTMUSP00000021174; RGD1563855; RP23-37L2.1; CCHL_HUMAN.   
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領(lǐng)域 心血管  信號轉(zhuǎn)導(dǎo)  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Dog, Horse, )
產(chǎn)品應(yīng)用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 31kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HCCS
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(chǎn)品介紹 background:
HCCS is a 79 amino acid protein that may act as a tumor suppressor, promoting the apoptosis of cancer cells. Expressed in leukocytes, lung, spleen, liver, heart, kidney, muscle and uterine cervix, HCCS-1 is down-regulated in cervical cancers. The gene encoding HCCS-1 maps to human chromosome 15q25.1. Encoding more than 700 genes, chromosome 15 is made up of approximately 106 million base pairs and comprises about 3% of the human genome. Angelman and Prader-Willi syndromes are associated with loss of function or deletion of genes in the 15q11-q13 region. Tay-Sachs disease is a lethal disorder associated with mutations of the HEXA gene, which is encoded by chromosome 15. Marfan syndrome is associated with chromosome 15 through the FBN1 gene.

Function:
Links covalently the heme group to the apoprotein of cytochrome c (By similarity).

Subcellular Location:
Mitochondrion inner membrane (Potential).

DISEASE:
Microphthalmia, syndromic, 7 (MCOPS7) [MIM:309801]: A disease characterized by unilateral or bilateral microphthalmia, linear skin defects in affected females, and in utero lethality for males. Skin defects are limited to the face and neck, consisting of areas of aplastic skin that heal with age to form hyperpigmented areas. Additional features in female patients include agenesis of the corpus callosum, sclerocornea, chorioretinal abnormalities, infantile seizures, congenital heart defect, mental retardation, and diaphragmatic hernia. Microphthalmia is a disorder of eye formation, ranging from small size of a single eye to complete bilateral absence of ocular tissues (anophthalmia). In many cases, microphthalmia/anophthalmia occurs in association with syndromes that include non-ocular abnormalities. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the cytochrome c-type heme lyase family.
Contains 2 HRM (heme regulatory motif) repeats.

Database links:

Entrez Gene: 3052 Human

Entrez Gene: 15159 Mouse

Entrez Gene: 317444 Rat

Omim: 300056 Human

SwissProt: P53701 Human

SwissProt: P53702 Mouse

Unigene: 211571 Human

Unigene: 284033 Mouse

Unigene: 162316 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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