免疫原
The antiserum was produced against synthesized peptide derived from human Cytochrome c-type Heme Lyase. AA range:81-130
特异性
HCCS Polyclonal Antibody detects endogenous levels of HCCS protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/20000. Not yet tested in other applications.
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
holocytochrome c synthase(HCCS) Homo sapiens The protein encoded by this gene is an enzyme that covalently links a heme group to the apoprotein of cytochrome c. Defects in this gene are a cause of microphthalmia syndromic type 7 (MCOPS7). Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jan 2010],
功能
catalytic activity:Holocytochrome c = apocytochrome c + heme.,disease:Defects in HCCS are a cause of microphthalmia syndromic type 7 (MCOPS7) [MIM:309801]; also known as microphthalmia with linear skin defects (MLS) or MIDAS syndrome. Microphthalmia is a clinically heterogeneous 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. MCOPS7 is a disorder 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, scle
蛋白名称
Cytochrome c-type heme lyase
其他名称
HCCS; CCHL; Cytochrome c-type heme lyase; CCHL; Holocytochrome c-type synthase
Fields
>>Porphyrin metabolism;>>Metabolic pathways
细胞定位
Mitochondrion inner membrane . Membrane ; Lipid-anchor .
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