HSCB - HscB mitochondrial iron-sulfur cluster cochaperone Gene

Homo sapiens

Also known as JAC1; HSC20; SIDBA5; DNAJC20

Gene ID: 150274 | Gene type: protein coding

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	Reference	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	Reference	Source
involved in iron-sulfur cluster assembly	IMP IMP: Inferred from mutant phenotype	20668094	GOA

Cellular Component GO Annotation	Evidence	Reference	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 Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
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

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS06386	HSCB Human Pre-designed siRNA Set A	Pre-designed Sets	/	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

Name
Email *

	Sorry, but the email address you supplied was invalid.