HSCB - HscB mitochondrial iron-sulfur cluster cochaperone Gene

Also Known as JAC1; HSC20; SIDBA5; DNAJC20

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 150274

About HSCB

Cytogenetic location: 22q12.1 Genomic coordinates (GRCh38): 22:28,742,032-28,757,510 (from NCBI)

Ubiquitous expression in ovary (RPKM 4.7), prostate (RPKM 4.7) and 25 other tissues.

Summary

This gene encodes a DnaJ-type co-chaperone and member of the heat shock cognate B (HscB) family of proteins. The encoded protein plays a role in the synthesis of iron-sulfur clusters, protein cofactors that are involved in the redox reactions of mitochondrial electron transport and Other processes. Cells in which this gene is knocked down exhibit reduced activity of iron-sulfur cluster-dependent Enzymes including Succinate Dehydrogenase and aconitase. The encoded protein may stimulate the ATPase activity of the mitochondrial stress-70 protein. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2015]

HSCB Products (5)

mRNA	Protein	Name
NM_001318314.2	NP_001305243.1	iron-sulfur cluster co-chaperone protein HscB isoform 2 precursor
NM_001318315.2	NP_001305244.1	iron-sulfur cluster co-chaperone protein HscB isoform 3 precursor
NM_001318316.2	NP_001305245.1	iron-sulfur cluster co-chaperone protein HscB isoform 4
NM_001363856.1	NP_001350785.1	iron-sulfur cluster co-chaperone protein HscB isoform 3 precursor
NM_172002.5	NP_741999.3	iron-sulfur cluster co-chaperone protein HscB isoform 1 precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Verweise	Source
enables identical protein binding	IPI IPI: Inferred from physical interaction	24606901	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	20668094	GOA

Biological Process GO Annotation	Evidence	Verweise	Source
involved in iron-sulfur cluster assembly	IMP IMP: Inferred from mutant phenotype	20668094	GOA

Cellular Component GO Annotation	Evidence	Verweise	Source
located in cytoplasm	IDA IDA: Inferred from direct assay	20668094	GOA
located in mitochondrion	IDA IDA: Inferred from direct assay	20668094	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

HSCB Protein Structure

DnaJ

HSCB_C

0
100
200
235 a.a.

Protein Preferred Names	Protein Names
iron-sulfur cluster co-chaperone protein HscB	DnaJ (Hsp40) homolog, subfamily C, member 20

HSCB Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Verweise
Intra	HSCB	Q8IWL3	GLRX5	Homo sapiens	Q86SX6	Anti Bait CoIP	24606901
Intra	HSCB	Q8IWL3	GLRX5	Homo sapiens	Q86SX6	Anti Tag CoIP	24606901
Intra	HSCB	Q8IWL3	GLRX5	Homo sapiens	Q86SX6	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	ETFA	Homo sapiens	P13804	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	ETFA	Homo sapiens	P13804	Anti Tag CoIP	28380382
Intra	HSCB	Q8IWL3	ETFA	Homo sapiens	P13804	Y2H	24606901
Intra	HSCB	Q8IWL3	SDHB	Homo sapiens	P21912	Y2H	24606901
Intra	HSCB	Q8IWL3	SDHB	Homo sapiens	P21912	Anti Tag CoIP	24606901
Intra	HSCB	Q8IWL3	SDHB	Homo sapiens	P21912	Anti Bait CoIP	24606901
Intra	HSCB	Q8IWL3	SDHB	Homo sapiens	P21912	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	SDHB	Homo sapiens	P21912	Anti Tag CoIP	28380382
Intra	HSCB	Q8IWL3	SDHAF1	Homo sapiens	A6NFY7	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	SDHAF1	Homo sapiens	A6NFY7	Anti Tag CoIP	24606901
Intra	HSCB	Q8IWL3	HELZ	Homo sapiens	P42694	Anti Tag CoIP	24606901
Intra	HSCB	Q8IWL3	HELZ	Homo sapiens	P42694	Y2H	24606901
Intra	HSCB	Q8IWL3	HELZ	Homo sapiens	P42694	Anti Bait CoIP	24606901
Intra	HSCB	Q8IWL3	LYRM7	Homo sapiens	Q5U5X0	Anti Bait CoIP	24606901
Intra	HSCB	Q8IWL3	LYRM7	Homo sapiens	Q5U5X0	Anti Tag CoIP	28380382
Intra	HSCB	Q8IWL3	LYRM7	Homo sapiens	Q5U5X0	Y2H	24606901
Intra	HSCB	Q8IWL3	LYRM7	Homo sapiens	Q5U5X0	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	SUCLG2	Homo sapiens	Q96I99	Y2H	24606901
Intra	HSCB	Q8IWL3	SUCLG2	Homo sapiens	Q96I99	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	SUCLG2	Homo sapiens	Q96I99	Anti Tag CoIP	28380382
Intra	HSCB	Q8IWL3	SUCLG2	Homo sapiens	Q96I99	Anti Bait CoIP	24606901
Intra	HSCB	Q8IWL3	HSPA9	Homo sapiens	P38646	Anti Tag CoIP	28380382
Intra	HSCB	Q8IWL3	HSPA9	Homo sapiens	P38646	Anti Tag CoIP	24606901
Intra	HSCB	Q8IWL3	HSPA9	Homo sapiens	P38646	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	HSPA9	Homo sapiens	P38646	Anti Bait CoIP	24606901
Intra	HSCB	Q8IWL3	EPRS1	Homo sapiens	P07814	Y2H	24606901
Intra	HSCB	Q8IWL3	EPRS1	Homo sapiens	P07814	Anti Tag CoIP	24606901
Intra	HSCB	Q8IWL3	EPRS1	Homo sapiens	P07814	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	NQO2	Homo sapiens	P16083	Y2H	24606901
Intra	HSCB	Q8IWL3	NQO2	Homo sapiens	P16083	Anti Bait CoIP	24606901
Intra	HSCB	Q8IWL3	NQO2	Homo sapiens	P16083	Anti Tag CoIP	24606901
Intra	HSCB	Q8IWL3	NDUFV2	Homo sapiens	P19404	Anti Bait CoIP	28380382
Intra	HSCB	Q8IWL3	NDUFV2	Homo sapiens	P19404	Anti Tag CoIP	28380382
Intra	HSCB	Q8IWL3	AGTRAP	Homo sapiens	Q6RW13	Validated Y2H	25416956

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases	Alias
Anemia, Sideroblastic, 5	SIDBA5
Sideroblastic Anemia	Anemia Sideroblastic Anemia, Sideroblastic Anemia, Hypochromic With Iron Loading
Epilepsy, Juvenile Absence 1	Epilepsy, Juvenile Absence, Susceptibility To, 1 EJA1 JAE1 Juvenile Absence Epilepsy 1 Susceptibility To Juvenile Absence Epilepsy 1 Epilepsy, Juvenile Absence, Susceptibility To, Type 1 Absence Epilepsy
Multiple Mitochondrial Dysfunctions Syndrome 1	MMDS1 Mmds Nfu1 Deficiency Multiple Mitochondrial Dysfunctions Syndrome Type 1 Mitochondrial Dysfunctions Syndrome, Multiple, Type 1
Combined Oxidative Phosphorylation Deficiency 19	COXPD19 Severe Neonatal Lactic Acidosis Due To Nfs1-Isd11 Complex Deficiency Combined Oxidative Phosphorylation Deficiency, Type 19
Pica Disease	Pica
Anemia, Sideroblastic, And Spinocerebellar Ataxia	X-Linked Sideroblastic Anemia With Ataxia ASAT X-Linked Sideroblastic Anemia And Ataxia X-Linked Sideroblastic Anemia And Spinocerebellar Ataxia Anemia, Sideroblastic, With Ataxia Anemia Sideroblastic And Spinocerebellar Ataxia Pagon Bird Detter Syndrome Pagon-Bird-Detter Syndrome Xlsa-A X-Linked Sideroblastic Anaemia And Ataxia X-Linked Sideroblastic Anaemia With Ataxia Sideroblastic Anemia With Spinocerebellar Ataxia Xlsa/A Anemia, Sideroblastic, Spinocerebellar Ataxia Sideroblastic Anemia And Ataxia Anemia Sideroblastic, And Spinocerebellar Ataxia
Anemia, Sideroblastic, 1	Xlsa X-Linked Sideroblastic Anemia Hypochromic Anemia Anh1 Hereditary Iron-Loading Anemia Anemia, Sideroblastic, X-Linked Anemia, Hereditary Sideroblastic Erythroid 5-Aminolevulinate Synthase Deficiency Hereditary Sideroblastic Anemia SIDBA1 Anemia, Hypochromic Sideroblastic Anemia 1 Anemia Hypochromic X Chromosome-Linked Sideroblastic Anemia Sideroblastic Anaemia 1 X-Linked Sideroblastic Anaemia Anemia Hereditary Sideroblastic Anemia Sex-Linked Hypochromic Sideroblastic Congenital Sideroblastic Anemia Sideroblastic Anemia X-Linked Anemia, Sex-Linked Hypochromic Sideroblastic Congenital Sideroblastic Anaemia X-Linked Pyridoxine-Responsive Sideroblastic Anemia Anemia Congenital Sideroblastic Anemia, Sideroblastic, Type 1 Sex-Linked Hypochromic Sideroblastic Anaemia Autosomal Recessive Sideroblastic Anaemia Familial Sex Linked Hypochromic Anaemia
Sideroblastic Anemia With B-Cell Immunodeficiency, Periodic Fevers, And Developmental Delay	SIFD Congenital Sideroblastic Anemia-B-Cell Immunodeficiency-Periodic Fever-Developmental Delay Syndrome Sifd Syndrome
Multiple Mitochondrial Dysfunctions Syndrome	Fatal Multiple Mitochondrial Dysfunctions Syndrome Fatal Multiple Mitochondrial Dysfunction Syndrome Mmds Multiple Mitochondrial Dysfunction Syndrome Mitochondrial Dysfunctions, Multiple, Syndrome Multiple Mitochondrial Dysfunctions Syndrome 1
Protoporphyria, Erythropoietic, 1	Erythropoietic Protoporphyria Epp Ferrochelatase Deficiency Protoporphyria, Erythropoietic Erythrohepatic Protoporphyria Heme Synthetase Deficiency Autosomal Erythropoietic Protoporphyria EPP1 Protoporphyria Protoporphyria Erythropoietic
Autosomal Recessive Cerebellar Ataxia	Arca
Bardet-Biedl Syndrome	Bbs Biedl-Bardet Syndrome

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 (3)

Art. -Nr.	Produktname	Wirkung	Reinheit	Rare Diseases
HY-RS06386	HSCB Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS22204	Hscb Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS28726	Hscb Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Felis catus	HSCB	VGNC	VGNC:107710
Macaca mulatta	HSCB	VGNC	VGNC:73454
Mus musculus	HSCB	MGD	MGI:2141135
Canis familiaris	HSCB	VGNC	VGNC:41796
Rattus norvegicus	HSCB	RGD	RGD:1311005
Bos taurus	HSCB	VGNC	VGNC:29964
Others	HSCB	NCBI