ITT2107
ITT2107
- Catalog: ITT2107
- Gene/Protein: HCCS
- Product Description: Immunotag™ HCCS Polyclonal Antibody
385.0000
Price in reward points: 385
| Antibody Specification | |
| Datasheet | 
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| Target Protein | HCCS |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | WB,IHC-p,IF,ELISA |
| Recommended Dilution | 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. |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse,Monkey |
| Host Species | Rabbit |
| Immunogen | The antiserum was produced against synthesized peptide derived from human Cytochrome c-type Heme Lyase. AA range:81-130 |
| Specificity | HCCS Polyclonal Antibody detects endogenous levels of HCCS protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Gene Name | HCCS |
| Accession No. | P53701 P53702 |
| Alternate Names | HCCS; CCHL; Cytochrome c-type heme lyase; CCHL; Holocytochrome c-type synthase |
| Description | 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], |
| Cell Pathway/ Category | Porphyrin and chlorophyll metabolism, |
| Protein Expression | Brain,Liver,Ovary, |
| Subcellular Localization | mitochondrion,mitochondrial inner membrane, |
| Protein Function | 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, sclerocornea, chorioretinal abnormalities, infantile seizures, congenital heart defect, mental retardation, and diaphragmatic hernia.,function:Links covalently the heme group to the apoprotein of cytochrome c.,similarity:Belongs to the cytochrome c-type heme lyase family.,similarity:Contains 2 HRM (heme regulatory motif) repeats., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |
| Material Safety Data Sheet | |
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