DNAJB1 - DnaJ heat shock protein family (Hsp40) member B1 Gene

Homo sapiens

Also known as Hdj1; Sis1; HSPF1; Hsp40; RSPH16B

Gene ID: 3337 | Gene type: protein coding

About DNAJB1

Cytogenetic location: 19p13.12 Genomic coordinates (GRCh38): 19:14,514,769-14,560,391 (from NCBI)

This gene has 25 transcripts (splice variants), 425 orthologues, 11 paralogues and is associated with 55 phenotypes. Ubiquitous expression in esophagus (RPKM 87.8), adrenal (RPKM 84.6) and 24 other tissues.

Summary

This gene encodes a member of the DnaJ or HSP40 (heat shock protein 40 kD) family of proteins. DNAJ family members are characterized by a highly conserved amino acid stretch called the 'J-domain' and function as one of the two major classes of molecular chaperones involved in a wide range of cellular events, such as protein folding and oligomeric protein complex assembly. The encoded protein is a molecular chaperone that stimulates the ATPase activity of HSP70 heat-shock proteins in order to promote protein folding and prevent misfolded protein aggregation. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Sep 2015]

DNAJB1 Products(7)

mRNA	Protein	Name
XM_011527956.4	XP_011526258.1	dnaJ homolog subfamily B member 1 isoform X1
NM_006145.3	NP_006136.1	dnaJ homolog subfamily B member 1 isoform 1
NM_001300914.2	NP_001287843.1	dnaJ homolog subfamily B member 1 isoform 2
NM_001313964.2	NP_001300893.1	dnaJ homolog subfamily B member 1 isoform 2
XM_047438746.1	XP_047294702.1	dnaJ homolog subfamily B member 1 isoform X1
XM_006722733.3	XP_006722796.1	dnaJ homolog subfamily B member 1 isoform X1
XM_047438745.1	XP_047294701.1	dnaJ homolog subfamily B member 1 isoform X1

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables ATPase activator activity	IDA IDA: Inferred from direct assay	20060297	GOA
enables ATPase binding	IPI IPI: Inferred from physical interaction	23921388	GOA
enables Hsp70 protein binding	IPI IPI: Inferred from physical interaction	23921388	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	9499401	GOA
enables protein-folding chaperone binding	IPI IPI: Inferred from physical interaction	21231916	GOA
enables transcription corepressor activity	IDA IDA: Inferred from direct assay	9499401	GOA
enables transcription regulator inhibitor activity	IDA IDA: Inferred from direct assay	9499401	GOA
enables unfolded protein binding	IDA IDA: Inferred from direct assay	21231916	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in cellular response to heat	IDA IDA: Inferred from direct assay	9499401	GOA
involved in chaperone cofactor-dependent protein refolding	IDA IDA: Inferred from direct assay	18620420	GOA
involved in negative regulation of inclusion body assembly	IDA IDA: Inferred from direct assay	21231916	GOA
involved in negative regulation of transcription by RNA polymerase II	IDA IDA: Inferred from direct assay	9499401	GOA
involved in positive regulation of ATP-dependent activity	IDA IDA: Inferred from direct assay	23921388	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
located in cytoplasm	IDA IDA: Inferred from direct assay	25468996	GOA
located in cytosol	IDA IDA: Inferred from direct assay	21231916	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

DNAJB1 Protein Structure

DnaJ

DnaJ_C

0
100
200
300
340 a.a.

Protein Preferred Names

Protein Names

dnaJ homolog subfamily B member 1

DnaJ (Hsp40) homolog, subfamily B, member 1	dnaJ protein homolog 1
heat shock 40 kDa protein 1	human DnaJ protein 1
radial spoke 16 homolog B

DNAJB1 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	DNAJB1	P25685	TBL1X	Homo sapiens	O60907-2	Y2H Pooling	32814053
Intra	DNAJB1	P25685	TBL1X	Homo sapiens	O60907-2	Validated Y2H	32814053
Intra	DNAJB1	P25685	TBL1X	Homo sapiens	O60907-2	Y2H Array	32814053
Intra	DNAJB1	P25685	UNG	Homo sapiens	P13051-2	Validated Y2H	32814053
Intra	DNAJB1	P25685	UNG	Homo sapiens	P13051-2	Y2H Pooling	32814053
Intra	DNAJB1	P25685	UNG	Homo sapiens	P13051-2	Y2H Array	32814053
Intra	DNAJB1	P25685	CDC37	Homo sapiens	Q16543	Anti Tag CoIP	35271311
Intra	DNAJB1	P25685	CDC37	Homo sapiens	Q16543	Y2H Array	21163940
Intra	DNAJB1	P25685	YWHAE	Homo sapiens	P62258	Crosslink	36931259
Intra	DNAJB1	P25685	TFF2	Homo sapiens	Q03403	Y2H Pooling	32814053
Intra	DNAJB1	P25685	TFF2	Homo sapiens	Q03403	Validated Y2H	32814053
Intra	DNAJB1	P25685	TFF2	Homo sapiens	Q03403	Y2H Array	32814053
Intra	DNAJB1	P25685	TERF1	Homo sapiens	P54274	Pull Down	21044950
Intra	DNAJB1	P25685	TTLL12	Homo sapiens	Q14166	Anti Tag CoIP	33961781
Intra	DNAJB1	P25685	TTLL12	Homo sapiens	Q14166	Anti Tag CoIP	28514442
Intra	DNAJB1	P25685	TTLL12	Homo sapiens	Q14166	Validated Y2H	32296183
Intra	DNAJB1	P25685	TTLL12	Homo sapiens	Q14166	Anti Tag CoIP	35271311

Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant DNAJB1 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P70331	HSP40/DNAJB1 Protein, Human (His)	P25685-1 (G2-I340)	≥95%

Related Diseases

Diseases

Alias

Fibrolamellar Carcinoma

Fibrolamellar Hepatocellular Carcinoma	Fhcc
Fibrolamellar Hepatocarcinoma	Hepatocellular Carcinoma, Fibrolamellar
Oncocytic Hepatocellular Tumor	Eosinophilic Glassy Cell Hepatoma
Eosinophilic Hepatocellular Carcinoma With Lamellar Fibrosis	Fl-Hcc
Fibrolamellar Oncocytic Hepatoma	Hepatocellular Carcinoma With Increased Stromal Fibrosis
Polygonal Cell Hepatocellular Carcinoma With Fibrous Stroma

Retinitis Pigmentosa

RP	Rod-Cone Dystrophy
Autosomal Recessive Retinitis Pigmentosa	Non-Syndromic Retinitis Pigmentosa
Pericentral Pigmentary Retinopathy	Pigmentary Retinopathy
Tapetoretinal Degeneration	Rcd
Retinitis Pigmentosa Autosomal Recessive	ARRP
Retinitis Pigmentosa, Autosomal Recessive	Retinitis Pigmentosa 1

Transvestism

Dual-Role Transvestism	Fetishistic Transvestism
Cross Dresser	Transvestic Fetishism

Nervous System Disease

Abnormality Of The Nervous System	Nervous System Diseases
Nervous System Disorder

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

Polyhydramnios

Cystic Fibrosis

Mucoviscidosis	CF
Pseudomonas Aeruginosa, Susceptibility To Chronic Infection By, In Cystic Fibrosis	Pseudomonas Aeruginosa Chronic Infection By, In Cystic Fibrosis
Cystic Fibrosis Lung Disease, Modifier Of	Cystic Fibrosis Of Pancreas
Fibrocystic Disease Of Pancreas	Cf - [Cystic Fibrosis]
Cystic Fibrosis Nos	Fibrocystic Disease
Fibrocystic Disease Of The Pancreas	Mucoviscidosis Of Pancreas
Nonproliferative Fibrocystic Disease	Pancreatic Cystic Fibrosis

Creutzfeldt-Jakob Disease

Variant Creutzfeldt-Jakob Disease	CJD
Bovine Spongiform Encephalopathy	Vcjd
Inherited Creutzfeldt-Jakob Disease	Creutzfeldt-Jakob Disease, Familial
Creutzfeldt Jakob Disease	Creutzfeldt-Jacob Disease
Creutzfeldt Jacob Disease	Sporadic Creutzfeldt-Jakob Disease
Encephalopathy, Bovine Spongiform	Creutzfeldt-Jakob Disease, Variant, Resistance To
Creutzfeldt-Jakob Disease, Variant	Creutzfeldt Jacob Syndrome
Jakob-Creutzfeldt Disease	Subacute Spongiform Encephalopathy
Transmissible Virus Dementia	New Variant Of Cjd
Nv-Cjd	Variant Cjd
Variant Creutzfeldt-Jacob Disease	Sporadic Cjd
Inherited Cjd	Acquired Creutzfeldt-Jakob Disease
Variant Mcj	Encephalopathy Bovine Spongiform
Familial Creutzfeldt-Jakob Disease	Creutzfeldt-Jakob Syndrome
New Variant Creutzfeldt-Jakob Disease	Creutzfeldt-Jakob Disease, Sporadic
Acquired Cjd	Scjd - [Sporadic Creutzfeldt-Jakob Disease]
Idiopathic Creutzfeldt-Jakob Disease	Creutzfeld-Jakob Disease Nos
Vcjd - [Variant Creutzfeldt-Jakob Disease]

Hepatocellular Carcinoma

Liver Cancer	Primary Liver Cancer
HCC	Hepatoma
Malignant Neoplasm Of Liver	Liver Neoplasms
Cancer, Hepatocellular	Liver Cell Carcinoma
Lcc	Hepatoblastoma, Somatic
Hepatic Cancer	Primary Malignant Neoplasm Of Liver
Rare Tumor Of Liver And Intrahepatic Biliary Tract	Hepatocellular Carcinoma, Somatic
Hepatocellular Carcinoma, Childhood Type, Somatic	Hepatocellular Cancer, Somatic
Ca Liver - Primary	Hepatic Neoplasm
Malignant Hepato-Biliary Neoplasm	Malignant Neoplasm Of Liver, Not Specified As Primary Or Secondary
Malignant Neoplasm Of Liver, Primary	Malignant Tumor Of Liver
Neoplasm Of Liver	Non-Resectable Primary Hepatic Malignant Neoplasm
Resectable Malignant Neoplasm Of Liver	Resectable Malignant Neoplasm Of The Liver
Primary Liver Carcinoma	Primary Malignant Liver Neoplasm
Primary Cancer Of Liver	Primary Tumor Of The Liver
Rare Tumor Of Liver And Ibt	Hepatocellular Cancer
Neoplasm Of The Liver	Hepatomas
Liver Neoplasm	Liver Carcinoma
Liver And Intrahepatic Biliary Tract Carcinoma	Malignant Hepatobiliary Neoplasm
Adult Primary Hepatocellular Carcinoma	Hepatoblastoma
Carcinoma Of Liver	Malignant Liver Tumour
Malignant Hepatic Tumour

Autosomal Dominant Cerebellar Ataxia

Spinocerebellar Ataxia	Adca
Pierre Marie Cerebellar Ataxia	Ataxia, Spinocerebellar
Sca	Autosomal Dominant Spinocerebellar Ataxia
Spinocerebellar Ataxias

Spinal Muscular Atrophy

Sma	5q Sma
Proximal Sma	Sma-Associated Sma
Spinal Amyotrophies	Spinal Amyotrophy
Spinal Muscle Degeneration	Spinal Muscle Wasting
Muscular Atrophy Spinal	Atrophy, Muscular, Spinal
Hereditary Motor Neuronopathy	Progressive Muscular Atrophy
Sma - [Spinal Muscular Atrophy]

Muscular Dystrophy, Limb-Girdle, Autosomal Dominant 1

Muscular Dystrophy, Limb-Girdle, Type 1e	Lgmd1d
Autosomal Dominant Limb-Girdle Muscular Dystrophy Type 1d	LGMDD1
Lgmd1e	Autosomal Dominant Limb-Girdle Muscular Dystrophy Type 1
Limb-Girdle Muscular Dystrophy Type 1d	Muscular Dystrophy, Limb-Girdle, Type 1d
Muscular Dystrophy, Limb-Girdle, Type 1d, Formerly	Lgmd1d, Formerly
Autosomal Dominant Limb-Girdle Muscular Dystrophy Type 1e	Muscular Dystrophy Limb-Girdle Type 1d
Muscular Dystrophy Limb-Girdle Type 1e	Dnajb6-Related Limb-Girdle Muscular Dystrophy D1
Dnajb6-Related Lgmd D1	Lgmd Type 1d
Limb-Girdle Muscular Dystrophy 1e	Limb-Girdle Muscular Dystrophy-1d, Autosomal Dominant
Dystrophy, Muscular, Limb-Girdle, Autosomal Dominant, Type 1	Dystrophy, Muscular, Limb-Girdle, Type 1e

Craniotubular Dysplasia, Ikegawa Type

CTDI	Craniotubular Dysplasia Ikegawa Type
Doid:0112340

Spinal And Bulbar Muscular Atrophy, X-Linked 1

Kennedy Disease	Sbma
Spinal And Bulbar Muscular Atrophy	Kennedy'S Disease
X-Linked Spinal And Bulbar Muscular Atrophy	SMAX1
Kd	Kennedy Spinal And Bulbar Muscular Atrophy
Spinobulbar Muscular Atrophy	Bulbospinal Muscular Atrophy, X-Linked
Bulbospinal Neuronopathy, X-Linked Recessive	Xbsn
Spinal And Bulbar Muscular Atrophy Of Kennedy	Bulbospinal Muscular Atrophy
X-Linked Bulbospinal Amyotrophy	Bulbo-Spinal Atrophy, X-Linked
Spinal Bulbar Muscular Atrophy	X-Linked Bulbo-Spinal Atrophy
X-Linked Spinal Bulbar Muscular Atrophy	X-Linked Bsma
X-Linked Bulbospinal Muscular Atrophy	Spinal And Bulbar Muscular Atrophy X-Linked 1
Bulbospinal Muscular Atrophy X-Linked	Bulbospinal Neuronopathy X-Linked Recessive
Kennedy Disease)	Kennedy Syndrome
Atrophy, Muscular, Spinal And Bulbar, Kennedy Type	Atrophy, Muscular, Spinobulbar
Bulbospinal Neuronopathy

Mixed Fibrolamellar Hepatocellular Carcinoma

Retinitis Pigmentosa 58

RP58	Retinitis Pigmentosa, Type 58

Craniodiaphyseal Dysplasia

Schaefer Stein Oshman Syndrome	CDD
Craniodiaphyseal Dysplasia, Dominant	Dominantly Inherited Craniodiaphyseal Dysplasia

Autosomal Dominant Limb-Girdle Muscular Dystrophy

Muscular Dystrophy, Limb-Girdle, Autosomal Dominant

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 (4)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-150072	DS89002333	Inhibitor	99.87%	Unkown
HY-RS03861	DNAJB1 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown
HY-RS03862	Dnajb1 Mouse Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown
HY-RS03863	Dnajb1 Rat Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Felis catus	DNAJB1	VGNC	VGNC:78440
Rattus norvegicus	DNAJB1	RGD	RGD:1304725
Macaca mulatta	DNAJB1	VGNC	VGNC:109616
Canis familiaris	DNAJB1	VGNC	VGNC:54594
Mus musculus	DNAJB1	MGD	MGI:1931874
Others	DNAJB1	NCBI

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