全细胞色素C合成酶抗体
规格：1mg/1ml
英文名： HCCS
别名： CCHL; DKFZp779I1858; EC 4.4.1.17; Holocytochrome c synthase (cytochrome c heme lyase); Holocytochrome c type synthase; MCOPS7; OTTHUMP00000022903; OTTHUMP00000022904; OTTHUMP00000022905; OTTMUSP000000
分子量： 31kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human HCCS
交叉反应：Human, Mouse, Rat, Dog, Horse,
细胞定位：
全细胞色素C合成酶抗体产品介绍：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,全细胞色素C合成酶抗体 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 全细胞色素C合成酶抗体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: UniProtKB/Swiss-Prot: P53701.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
全细胞色素C合成酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid