CCS - copper chaperone for superoxide dismutase Gene

Homo sapiens

Gene ID: 9973 | Gene type: protein coding

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	Reference	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	Reference	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

Sod_Cu

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 Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
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

Clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Phase
HY-111784	Inobrodib	Inhibitor	99.94%	Phase 2

Pre-clinical Phase

Bioactive Molecules (5)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-107636	DC_AC50	Inducer	99.97%	Unkown
HY-160005	LMP517	Inhibitor	99.58%	Unkown
HY-147373	DA-PROTAC		/	Unkown
HY-168202	CCS1477a	Control	/	Unkown
HY-RS02176	CCS Human Pre-designed siRNA Set A	Pre-designed Sets	/	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

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