HCCS Antibody

Code CSB-PA010165GA01HU
Size US$685
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Product Details

Uniprot No. P53701
Target Names HCCS
Alternative Names CCHL antibody; CCHL_HUMAN antibody; cytochrome c heme-lyase antibody; Cytochrome c-type heme lyase antibody; DKFZp779I1858 antibody; EC antibody; Hccs antibody; Holocytochrome c synthase (cytochrome c heme lyase) antibody; Holocytochrome c synthase antibody; Holocytochrome c type synthase antibody; Holocytochrome c-type synthase antibody; MCOPS7 antibody; OTTHUMP00000022903 antibody; OTTHUMP00000022904 antibody; OTTHUMP00000022905 antibody; OTTMUSP00000021173 antibody; OTTMUSP00000021174 antibody; RGD1563855 antibody; RP23-37L2.1 antibody
Raised in Rabbit
Species Reactivity Human,Mouse,Rat
Immunogen Human HCCS
Immunogen Species Homo sapiens (Human)
Isotype IgG
Purification Method Antigen Affinity purified
Concentration It differs from different batches. Please contact us to confirm it.
Buffer PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications ELISA,WB,IHC
Protocols ELISA Protocol
Western Blotting(WB) Protocol
Immunohistochemistry (IHC) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Target Data

Function Links covalently the heme group to the apoprotein of cytochrome c.
Gene References into Functions
  1. Data suggest that although HCCS mediates heme attachment to N-terminal cysteine in heme-attachment site (CXXXH) of cytochrome C variants, up to 50% of cytochrome C produced is modified in an oxygen-dependent manner, resulting in a mixed population of cytochrome c. [HCCS = holocytochrome c synthase] PMID: 28617588
  2. Bacterial cyt c biogenesis pathways (Systems I and II) appear to recognize simply the CXXCH motif, not requiring alpha helix-1. Results here explain mechanistically how HCCS (System III) requires an extended region adjacent to CXXCH for maturation. PMID: 27387500
  3. cysteines and histidine of the heme attachment site, Cys-XX-Cys-His play a key role in mitochondrial holocytochrome c synthase PMID: 25170082
  4. These data indicate that heme contacts mediated by residues within these domains modulate the dynamics of heme binding and contribute to the stability of the HCCS-heme-cytochrome c steady state ternary complex. PMID: 25054239
  5. Identification of a deletion in two patients, including HCCS, leads to the diagnosis of microphthalmia with linear skin defects syndrome PMID: 23401659
  6. Spectroscopic analyses of HCCS alone and complexes of HCCS with site-directed variants of cytochrome c revealed the fundamental steps of heme attachment and maturation. PMID: 23150584
  7. Through the study of genetically engineered mice, the loss of HCCS is demonstrated to cause the male lethality of microphthalmia with linear skin defects (MLS) syndrome. PMID: 12444108
  8. mutant proteins (R217C and Delta 197-268) were unable to complement a Saccharomyces cerevisiae mutant deficient for the HCCS orthologue Cyc3p, in contrast to wild-type HCCS PMID: 17033964
  9. Missense mutation p.E159K of HCCS, leading to loss-of-function of encoded holocytochrome c-type synthase, in female with microphthalmia of both eyes and bilateral sclerocornea may suggest HCCS as candidate for severe ocular manifestations. PMID: 17893649

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Involvement in disease Linear skin defects with multiple congenital anomalies 1 (LSDMCA1)
Subcellular Location Mitochondrion inner membrane, Membrane, Lipid-anchor
Protein Families Cytochrome c-type heme lyase family
Database Links

HGNC: 4837

OMIM: 300056

KEGG: hsa:3052

STRING: 9606.ENSP00000326579

UniGene: Hs.211571

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