HCCS Antibody

Code CSB-PA002862
Size US$100
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  • Western Blot analysis of COS7 cells using HCCS Polyclonal Antibody
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Product Details

Uniprot No.
Target Names
HCCS
Alternative Names
CCHL antibody; CCHL_HUMAN antibody; cytochrome c heme-lyase antibody; Cytochrome c-type heme lyase antibody; DKFZp779I1858 antibody; EC 4.4.1.17 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,Monkey
Immunogen
Synthesized peptide derived from the Internal region of Human HCCS.
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Isotype
IgG
Purification Method
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Form
Liquid
Tested Applications
WB, IHC, IF, ELISA
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:2000
IHC 1:100-1:300
IF 1:200-1:1000
ELISA 1:20000
Troubleshooting and 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.

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Target Background

Function
Lyase that catalyzes the covalent linking of the heme group to the cytochrome C apoprotein to produce the mature functional cytochrome.
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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7505 Fannin St., Ste 610, Room 7 (CUBIO Innovation Center), Houston, TX 77054, USA
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