HCCS Antibody (PACO23777)
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주문- SKU:
- PACO23777
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | HCCS Antibody (PACO23777) |
Antibody SKU: | PACO23777 |
Size: | 100ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC, IF |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:3000, IHC:1:50-1:100, IF:1:100-1:500 |
Species Reactivity: | Human, Mouse |
Immunogen: | Synthesized peptide derived from internal of human CCHL. |
Form: | Liquid |
Storage Buffer: | Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
Purification Method: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | Western blot analysis of extracts from COS7 cells, using Cytochrome c-type Heme Lyase antibody. |
![]() | Immunofluorescence analysis of MCF-7 cells, using Cytochrome c-type Heme Lyase antibody. |
![]() | Immunohistochemistry analysis of paraffin-embedded human tonsil tissue using Cytochrome c-type Heme Lyase antibody. |
Background: | Links covalently the heme group to the apoprotein of cytochrome c By similarity. |
Synonyms: | CCHL; cytochrome c-type heme lyase; EC 4.4.1.17; |
UniProt Protein Function: | HCCS: Links covalently the heme group to the apoprotein of cytochrome c. Defects in HCCS are a cause of microphthalmia syndromic type 7 (MCOPS7); 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. Belongs to the cytochrome c-type heme lyase family. |
UniProt Protein Details: | Protein type:Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Mitochondrial; EC 4.4.1.17; Lyase Chromosomal Location of Human Ortholog: Xp22.3 Cellular Component: mitochondrion; mitochondrial inner membrane Molecular Function:metal ion binding; holocytochrome-c synthase activity Biological Process: organ morphogenesis Disease: Microphthalmia, Syndromic 7 |
NCBI Summary: | 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] |
UniProt Code: | P53701 |
NCBI GenInfo Identifier: | 1705694 |
NCBI Gene ID: | 3052 |
NCBI Accession: | P53701.1 |
UniProt Secondary Accession: | P53701,Q502X8, B3KUS1, |
UniProt Related Accession: | P53701 |
Molecular Weight: | Observed MW: 31kDaCalculated MW 30kDa |
NCBI Full Name: | Cytochrome c-type heme lyase |
NCBI Synonym Full Names: | holocytochrome c synthase |
NCBI Official Symbol: | HCCS |
NCBI Official Synonym Symbols: | MLS; CCHL; MCOPS7 |
NCBI Protein Information: | cytochrome c-type heme lyase; cytochrome c heme-lyase; holocytochrome c-type synthase; microphthalamia with linear skin defects |
UniProt Protein Name: | Cytochrome c-type heme lyase |
UniProt Synonym Protein Names: | Holocytochrome c-type synthase |
Protein Family: | Cytochrome c-type heme lyase |
UniProt Gene Name: | HCCS |
UniProt Entry Name: | CCHL_HUMAN |
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