UBQLN4 - ubiquilin 4 Gene

Homo sapiens

Also known as A1U; A1Up; UBIN; CIP75; C1orf6

Gene ID: 56893 | Gene type: protein coding

About UBQLN4

Cytogenetic location: 1q22 Genomic coordinates (GRCh38): 1:156,031,247-156,053,798 (from NCBI)

This gene has 3 transcripts (splice variants), 235 orthologues and 5 paralogues. Ubiquitous expression in brain (RPKM 15.5), endometrium (RPKM 13.7) and 25 other tissues.

Summary

Enables K48-linked polyubiquitin modification-dependent protein binding activity and identical protein binding activity. Involved in cellular response to DNA damage stimulus; negative regulation of double-strand break repair via homologous recombination; and regulation of cellular catabolic process. Located in several cellular components, including autophagosome; nucleoplasm; and site of DNA damage. Part of protein-containing complex. Colocalizes with cytosolic Proteasome complex and nuclear Proteasome complex. [provided by Alliance of Genome Resources, Apr 2022]

UBQLN4 Products(2)

mRNA	Protein	Name
NM_001304342.2	NP_001291271.1	ubiquilin-4 isoform 2
NM_020131.5	NP_064516.2	ubiquilin-4 isoform 1

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables K48-linked polyubiquitin modification-dependent protein binding	IDA IDA: Inferred from direct assay	29666234	GOA
enables identical protein binding	IPI IPI: Inferred from physical interaction	11001934	GOA
enables polyubiquitin modification-dependent protein binding	IDA IDA: Inferred from direct assay	15280365	GOA
enables polyubiquitin modification-dependent protein binding	IPI IPI: Inferred from physical interaction	29666234	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	11001934	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in DNA damage response	IDA IDA: Inferred from direct assay	30612738	GOA
involved in negative regulation of autophagosome maturation	IMP IMP: Inferred from mutant phenotype	23459205	GOA
involved in negative regulation of double-strand break repair via homologous recombination	IDA IDA: Inferred from direct assay	30612738	GOA
involved in regulation of proteasomal ubiquitin-dependent protein catabolic process	IDA IDA: Inferred from direct assay	27113755	GOA
involved in regulation of proteasomal ubiquitin-dependent protein catabolic process	IMP IMP: Inferred from mutant phenotype	15280365	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
located in autophagosome	IDA IDA: Inferred from direct assay	23459205	GOA
located in cytoplasm	IDA IDA: Inferred from direct assay	11001934	GOA
located in cytosol	IDA IDA: Inferred from direct assay	29666234	GOA
part of cytosolic proteasome complex	IDA IDA: Inferred from direct assay	15280365	GOA
part of nuclear proteasome complex	IDA IDA: Inferred from direct assay	15280365	GOA
located in nucleus	IDA IDA: Inferred from direct assay	11001934	GOA
part of protein-containing complex	IDA IDA: Inferred from direct assay	29666234	GOA
located in site of DNA damage	IDA IDA: Inferred from direct assay	30612738	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

UBQLN4 Protein Structure

ubiquitin

UBA

0
100
200
300
400
500
601 a.a.

Protein Preferred Names

Protein Names

ubiquilin-4

ataxin-1 interacting ubiquitin-like protein

UBQLN4 Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	UBQLN4	Q9NRR5	PDLIM7	Homo sapiens	Q9NR12	Pull Down	16713569
Intra	UBQLN4	Q9NRR5	UBQLN1	Homo sapiens	Q9UMX0	Anti Tag CoIP	23459205
Intra	UBQLN4	Q9NRR5	UBQLN4	Homo sapiens	Q9NRR5	Y2H	11001934
Intra	UBQLN4	Q9NRR5	MAP1LC3A	Homo sapiens	Q9H492	Anti Tag CoIP	23459205
Intra	UBQLN4	Q9NRR5	UBQLN4	Homo sapiens	Q9NRR5	Anti Tag CoIP	23459205
Intra	UBQLN4	Q9NRR5	UBQLN1	Homo sapiens	Q9UMX0	Confocal	23459205
Intra	UBQLN4	Q9NRR5	UBQLN4	Homo sapiens	Q9NRR5	Y2H	16713569
Intra	UBQLN4	Q9NRR5	UBQLN1	Homo sapiens	Q9UMX0	Anti Bait CoIP	23459205
Intra	UBQLN4	Q9NRR5	ATXN1	Homo sapiens	P54253	Y2H	11001934
Intra	UBQLN4	Q9NRR5	UBQLN1	Homo sapiens	Q9UMX0	Y2H Array	16713569
Intra	UBQLN4	Q9NRR5	ATXN1	Homo sapiens	P54253	Y2H	16713569
Intra	UBQLN4	Q9NRR5	UBQLN1	Homo sapiens	Q9UMX0	Y2H	16713569
Intra	UBQLN4	Q9NRR5	ATXN1	Homo sapiens	P54253	IF	11001934
Intra	UBQLN4	Q9NRR5	FKBP2	Homo sapiens	P26885	Pull Down	16713569
Intra	UBQLN4	Q9NRR5	TRIM32	Homo sapiens	Q13049	Y2H Array	16713569
Intra	UBQLN4	Q9NRR5	RAI2	Homo sapiens	Q9Y5P3	Y2H Array	16713569
Intra	UBQLN4	Q9NRR5	RAI2	Homo sapiens	Q9Y5P3	Y2H	16713569
Intra	UBQLN4	Q9NRR5	ZNF205	Homo sapiens	O95201	Pull Down	16713569

Cross: Cross-species interaction Intra: Intraspecies interaction

Related Diseases

Diseases

Alias

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

Mumps

Parotitis Due To Mumps Virus	Mumps Nos
Epidemic Parotitis	Infectious Parotitis

Arthrogryposis, Distal, Type 1b

DA1B	Distal Arthrogryposis Type 1b
Arthrogryposis, Distal, 1b

Cataract 3, Multiple Types

Cataract 3 Multiple Types	CTRCT3
Cca2	Cataract, Congenital, Cerulean Type, 2
Cataract 3, Multiple Types, With Or Without Microcornea	Cataract 3 Multiple Types With Or Without Microcornea
Congenital Cerulean Type Cataract 2	Congenital Cataract Blue Dot Type 2
Congenital Cataract Cerulean Type 2	Cspc
Sutural Cataract With Punctate And Cerulean Opacities

Spinal Muscular Atrophy, X-Linked 2

SMAX2	Arthrogryposis Multiplex Congenita, Distal, X-Linked
Infantile-Onset X-Linked Spinal Muscular Atrophy	Xlsma
Amcx1	Spinal Muscular Atrophy, X-Linked Lethal Infantile
Spinal Muscular Atrophy, Infantile X-Linked	Amc, Distal, X-Linked
Spinal Muscular Atrophy, X-Linked 2, Infantile	X-Linked Spinal Muscular Atrophy 2
Spinal Muscular Atrophy With Arthrogryposis	X-Linked Distal Arthrogryposis Multiplex Congenita
X-Linked Spinal Muscular Atrophy Type 2	Xl-Sma
Arthrogryposis, X-Linked, Type I	Spinal Muscular Atrophy, X-Linked Infantile
X-Linked Infantile Spinal Muscular Atrophy	Arthrogryposis, X-Lined, Type I
Distal X-Linked Amc	Infantile X-Linked Sma
X-Linked Arthrogryposis Multiplex Congenita	X-Linked Arthrogryposis Type I
X-Linked Lethal Infantile Sma	Arthrogryposis Spinal Muscular Atrophy
Spinal Muscular Atrophy X-Linked 2	Amc Distal X-Linked
Arthrogryposis Multiplex Congenita Distal X-Linked	Arthrogryposis X-Linked Type I
Spinal Muscular Atrophy Infantile X-Linked	Spinal Muscular Atrophy X-Linked Lethal Infantile
Atrophy, Muscular, Spinal, X-Linked, Type 2, Infantile

Brown-Vialetto-Van Laere Syndrome

Spinocerebellar Ataxia 1

Spinocerebellar Ataxia Type 1	SCA1
Olivopontocerebellar Atrophy I	Opca1
Opca4	Menzel Type Opca
Schut-Haymaker Type Opca	Spinocerebellar Atrophy I
Opca I	Olivopontocerebellar Atrophy Iv
Opca Iv	Cerebelloparenchymal Disorder I
Cpd1	Olivopontocerebellar Atrophy 1
Cerebelloparenchymal Disorder 1	Olivopontocerebellar Atrophy 4
Spinocerebellar Atrophy 1	Type 1 Spinocerebellar Ataxia
Spinocerebellar Ataxia-1	Ataxia, Spinocerebellar, Type 1

Microcephaly

Microencephaly	Microcephalus
Microcephalic	Nanocephaly
Congenital Microcephaly	Brain Hypoplasia
Brain Nondevelopment	Cephalic Hypoplasia
Undeveloped Cerebrum	Undeveloped Brain
Micrencephalon	Micrencephaly

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-RS15385	UBQLN4 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Rattus norvegicus	UBQLN4	RGD	RGD:1308273
Mus musculus	UBQLN4	MGD	MGI:2150152
Macaca mulatta	UBQLN4	VGNC	VGNC:100182
Bos taurus	UBQLN4	VGNC	VGNC:55694
Felis catus	UBQLN4	VGNC	VGNC:66783
Canis familiaris	UBQLN4	VGNC	VGNC:48087

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