CCS - copper chaperone for superoxide dismutase Gene

Species: Homo sapiens

Gene Type: protein coding
Gene ID: 9973

About CCS

Cytogenetic location: 11q13.2 Genomic coordinates (GRCh38): 11:66,593,185-66,606,019 (from NCBI)

This gene has 9 transcripts (splice variants), 192 orthologues and 2 paralogues. Ubiquitous expression in liver (RPKM 18.8), spleen (RPKM 17.6) and 25 other tissues.

Summary

Copper chaperone for superoxide dismutase specifically delivers Cu to copper/zinc superoxide dismutase and may activate copper/zinc superoxide dismutase through direct insertion of the Cu cofactor. [provided by RefSeq, Jul 2008]

CCS Products (1)

mRNA Protein Name
NM_005125.2 NP_005116.1 copper chaperone for superoxide dismutase

Gene Ontology

  • Molecular Function
  • Cellular Component
Molecular Function GO Annotation Evidence References Source
enables copper ion binding IDA
IDA: Inferred from direct assay
9726962 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
9726962 GOA
Cellular Component GO Annotation Evidence References Source
located in cytoplasm IDA
IDA: Inferred from direct assay
9726962 GOA
located in nucleus IDA
IDA: Inferred from direct assay
9726962 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

CCS Protein Structure

HMA

HMA: Heavy-metal-associated domain (16 - 70)

Sod_Cu

Sod_Cu: Copper/zinc superoxide dismutase (SODC) (91 - 230)

  • 0
  • 100
  • 200
  • 274 a.a.
Protein Preferred Names Protein Names

copper chaperone for superoxide dismutase

  • superoxide dismutase copper chaperone

CCS Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method References
Intra
CCS O14618 XIAP Homo sapiens P98170
Anti Tag CoIP
22508683
Intra
CCS O14618 SOD1 Homo sapiens P00441
Anti Tag CoIP
22508683
Intra
CCS O14618 SOD1 Homo sapiens P00441
Pull Down
12968035
Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases Alias
Wilson Disease

  • Hepatolenticular Degeneration

  • WD

  • Wilson'S Disease

  • WND

  • Westphal-Strumpell Syndrome

  • Copper Storage Disease

  • Cerebral Pseudosclerosis

  • Westphal Pseudosclerosis

  • Hepatolenticular Degeneration Syndrome

  • Copper Retention

  • Hepatocerebral Degeneration

  • Kinnier-Wilson Disease

  • Neurohepatic Degeneration

  • Progressive Hepatolenticular Degeneration

  • Lenticular Degenerative Disease

  • Wilson'S Syndrome

  • Lenticular Syndrome
Amyotrophic Lateral Sclerosis 1

  • Amyotrophic Lateral Sclerosis

  • ALS

  • Lou Gehrig Disease

  • Amyotrophic Lateral Sclerosis Type 1

  • Charcot Disease

  • ALS1

  • Amyotrophic Lateral Sclerosis, Susceptibility To

  • Fals

  • Lou Gehrig'S Disease

  • Mnd

  • Motor Neuron Disease

  • Familial Amyotrophic Lateral Sclerosis

  • Amyotrophic Lateral Sclerosis 1, Familial

  • Amyotrophic Lateral Sclerosis 1, Autosomal Dominant

  • Motor Neuron Disease, Bulbar

  • Motor Neurone Disease

  • Amyotrophic Lateral Sclerosis With Dementia

  • Dementia With Amyotrophic Lateral Sclerosis

  • Motor Neuron Disease, Amyotrophic Lateral Sclerosis

  • Sclerosis, Lateral, Amyotrophic

  • Sclerosis, Lateral, Amyotrophic, Type 1

  • Amyotrophic Sclerosis

  • Als - [Amyotrophic Lateral Sclerosis]

  • Wasting Palsy

  • Amyotrophic Paralysis

  • Amyotrophy Lateral Sclerosis

  • Wasting Paralysis

  • Spinal Progressive Amyotrophy

  • Progressive Atrophic Paralysis
Menkes Disease

  • Copper Transport Disease

  • Menkes Syndrome

  • MNK

  • Kinky Hair Disease

  • Steely Hair Disease

  • Menkes Kinky-Hair Syndrome

  • Mk

  • Steely Hair Syndrome

  • Menkea Syndrome

  • Md

  • Menkes Kinky Hair Syndrome

  • Hypocupremia, Congenital

  • Kinky Hair Syndrome

  • X-Linked Copper Deficiency

  • Menkes Kinky Hair Disease
Aceruloplasminemia

  • Cerebellar Ataxia

  • Hypoceruloplasminemia

  • Hemosiderosis, Systemic, Due To Aceruloplasminemia

  • Familial Apoceruloplasmin Deficiency

  • Hereditary Ceruloplasmin Deficiency

  • Deficiency Of Ferroxidase

  • Hypoceruloplasminemia, Hereditary

  • Ceruloplasmin Deficiency

  • Systemic Hemosiderosis Due To Aceruloplasminemia

  • ACERULOP
Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Related Agents

  • Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (1)
Cat. No. Product Name
Effect
Purity
Rare Diseases
HY-184970 ATOX1 ligand-1 Inhibitor / Unkown

Orthologs Information

Species Symbol Source ID
Canis familiaris CCS VGNC VGNC:38919
Bos taurus CCS VGNC VGNC:26991
Mus musculus CCS MGD MGI:1333783
Rattus norvegicus CCS RGD RGD:620403
Felis catus CCS VGNC VGNC:81914
Macaca mulatta CCS VGNC VGNC:70744
Others CCS NCBI