CLPP - caseinolytic mitochondrial matrix peptidase proteolytic subunit Gene

Homo sapiens

Also known as DFNB81; PRLTS3

Gene ID: 8192 | Gene type: protein coding

About CLPP

Cytogenetic location: 19p13.3 Genomic coordinates (GRCh38): 19:6,361,531-6,370,242 (from NCBI)

This gene has 7 transcripts (splice variants), 196 orthologues and is associated with 3 phenotypes. Ubiquitous expression in kidney (RPKM 19.9), fat (RPKM 18.0) and 25 other tissues.

Summary

The protein encoded by this gene belongs to the peptidase family S14 and hydrolyzes proteins into small Peptides in the presence of ATP and magnesium. The protein is transported into mitochondrial matrix and is associated with the inner mitochondrial membrane. [provided by RefSeq, Jul 2008]

CLPP Products(1)

mRNA	Protein	Name
NM_006012.4	NP_006003.1	ATP-dependent Clp protease proteolytic subunit, mitochondrial precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables endopeptidase activity	IDA IDA: Inferred from direct assay	22354088	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	11923310	GOA
enables serine-type endopeptidase activity	IDA IDA: Inferred from direct assay	11923310	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in membrane protein proteolysis	IDA IDA: Inferred from direct assay	22354088	GOA
involved in proteolysis	IDA IDA: Inferred from direct assay	15522782	GOA
involved in proteolysis involved in protein catabolic process	IDA IDA: Inferred from direct assay	11923310	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
part of endopeptidase Clp complex	IDA IDA: Inferred from direct assay	11923310	GOA
part of endopeptidase Clp complex	IPI IPI: Inferred from physical interaction	15522782	GOA
located in mitochondrial matrix	IDA IDA: Inferred from direct assay	10525407	GOA
located in mitochondrion	IDA IDA: Inferred from direct assay	22354088	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

CLPP Protein Structure

CLP_protease

0
100
200
277 a.a.

Protein Preferred Names

Protein Names

ATP-dependent Clp protease proteolytic subunit, mitochondrial

ATP-dependent protease ClpAP, proteolytic subunit, human

CLPP Protein-protein interaction Information

Type Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
Intra	CLPP	Q16740	P4HA3	Homo sapiens	Q7Z4N8	Validated Y2H	32296183
Intra	CLPP	Q16740	TNPO3	Homo sapiens	Q9Y5L0	Validated Y2H	32296183
Intra	CLPP	Q16740	ABI2	Homo sapiens	Q9NYB9-2	Validated Y2H	32296183
Intra	CLPP	Q16740	C14orf119	Homo sapiens	Q9NWQ9	Validated Y2H	32296183
Intra	CLPP	Q16740	RBPMS	Homo sapiens	Q93062-3	Validated Y2H	32296183
Intra	CLPP	Q16740	DTX2	Homo sapiens	Q86UW9	Validated Y2H	32296183
Intra	CLPP	Q16740	PLSCR4	Homo sapiens	Q9NRQ2	Validated Y2H	32296183

Cross: Cross-species interaction Intra: Intraspecies interaction

CLPP Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P81930	CLPP Antibody (YA1675)	WB, IHC-P, IP	Human, Mouse, Rat

Related Diseases

Diseases

Alias

Perrault Syndrome 3

PRLTS3	Deafness, Autosomal Recessive 81, Formerly
Dfnb81, Formerly	Perrault Syndrome, Type 3

Perrault Syndrome

Gonadal Dysgenesis, Xx Type, With Deafness	Ovarian Dysgenesis With Sensorineural Deafness
Gonadal Dysgenesis, Xx Type	Gonadal Dysgenesis With Auditory Dysfunction, Autosomal Recessive Inheritance
Gonadal Dysgenesis With Sensorineural Deafness, Autosomal Recessive Inheritance	Xx Gonodal Dysgenesis-Deafness Syndrome
Xx Gonodal Dysgenesis-Hearing Loss Syndrome	Gonadal Dysgenesis Xx Type Deafness

Codas Syndrome

Cerebral, Ocular, Dental, Auricular, And Skeletal Anomalies Syndrome	Cerebral, Ocular, Dental, Auricular, And Skeletal Syndrome
Cerebro-Oculo-Dento-Auriculo-Skeletal Syndrome	Cerebrooculodentoauriculoskeletal Syndrome
CODASS	Cerebral, Ocular, Dental, Auricular, Skeletal Syndrome

Acute Laryngopharyngitis

Pharyngolaryngitis	Laryngopharyngitis
Hypopharyngitis

Learning Disability

Learning Disabilities	Learning Disorders
Academic Skill Disorder	Learning Disorder

Retinitis Pigmentosa 36

RP36	Retinitis Pigmentosa-36
Retinitis Pigmentosa, Type 36

Hereditary Spastic Paraplegia

Familial Spastic Paraplegia	Hereditary Spastic Paraparesis
Strumpell-Lorrain Disease	Familial Spastic Paraparesis
Hsp	Spg
Strümpell-Lorrain Disease	Spastic Paraplegia, Hereditary
French Settlement Disease	Strumpell-Lorrain Syndrome
Fsp	Spastic Paraplegia, Familial
Spastic Paraplegia Hereditary	Spastic Paraplegia 3, Autosomal Dominant
Spastic Paraparesis	Hereditary Spastic Paralysis
Familial Spastic Paralysis	Hereditary Spastic Ataxia

Pyosalpinx

Pyosalpingitis

Multiple Synostoses Syndrome

Symphalangism-Brachydactyly Syndrome	Deafness-Hermann Type Symphalangism Syndrome
Facio-Audio-Symphalangism	Hearing Loss-Hermann Type Symphalangism Syndrome
Wl Syndrome	Multiple Synostosis Syndrome

Myasthenic Syndrome, Congenital, 12

Congenital Myasthenic Syndrome 12	CMS12
Myasthenic Syndrome, Congenital, With Tubular Aggregates 1	Cmsta1
Myasthenia, Congenital, 12, With Tubular Aggregates	Congenital Myasthenia 12 With Tubular Aggregates
Limb-Girdle Myasthenia With Tubular Aggregates	Myasthenia, Congenital, With Tubular Aggregates 1
Myasthenic Syndrome, Congenital, With Tubular Aggregates, 1

46 Xx Gonadal Dysgenesis

Ovarian Dysgenesis	Gonadal Dysgenesis, 46,Xx
Dysgenesis, Ovarian

Intellectual Developmental Disorder, Autosomal Dominant 30, With Speech Delay And Behavioral Abnormalities

MRD30	Mental Retardation, Autosomal Dominant 30
Intellectual Developmental Disorder, Autosomal Dominant 30	Autosomal Dominant Non-Syndromic Intellectual Disability 30
Autosomal Dominant Intellectual Developmental Disorder 30	Autosomal Dominant Mental Retardation 30
Mental Retardation, Autosomal Dominant, Type 30

Premature Menopause

Primary Ovarian Insufficiency	Premature Ovarian Failure
Hypergonadotropic Hypogonadism	Premature Ovarian Insufficiency
Menopause - Premature	Menopause Praecox
Menopause Premature	Menopause, Premature
Female Hypergonadotropic Hypogonadism	Hypergonadotrophic Ovarian Failure
Primary Female Hypogonadism	Pof - [Premature Ovarian Failure]
Ovarian Failure	Ovarian Secretion Suppression
Ovary Hyposecretion	Ovary Secretion Deficiency
Premature Menopause Nos

Mitochondrial Dna Depletion Syndrome 7

Ohaha Syndrome	Infantile Onset Spinocerebellar Ataxia
Iosca	Infantile-Onset Spinocerebellar Ataxia
Spinocerebellar Ataxia 8	MTDPS7
Ophthalmoplegia, Hypotonia, Ataxia, Hypacusis, And Athetosis	Ophthalmoplegia-Hypotonia-Ataxia-Hypoacusis-Athetosis Syndrome
Sca8	Spinocerebellar Ataxia Infantile With Sensory Neuropathy
Spinocerebellar Ataxia, Infantile-Onset	Ophthalmoplegia, Hypotonia, Ataxia, Hypoacusis, And Athetosis
Spinocerebellar Ataxia, Infantile, With Sensory Neuropathy	Spinocerebellar Ataxia 8, Formerly
Sca8, Formerly	Iosca, Mitochondrial Dna Depletion Syndrome 7
Ophthalmoplegia - Hypotonia - Ataxia - Hypoacusis - Athetosis	Mitochondrial Dna Depletion Syndrome, Hepatocerebrorenal Form
Mtdna Depletion Syndrome, Hepatocerebrorenal Form	Mitochondrial Dna Depletion Syndrome 7 Hepatocerebral Type
Ophthalmoplegia Hypotonia Ataxia Hypoacusis And Athetosis	Pure Spinocerebellar Ataxia Japanese Type
Sca4 Pure Japanese Type	Spinocerebellar Ataxia Infantile-Onset
Mitochondrial Dna Depletion Syndrome , Type 7

Sensorineural Hearing Loss

Sensory Hearing Loss	Sensorineural Deafness
Sensorineural Hearing Loss Disorder	Hearing Loss, Sensorineural
Central Hearing Loss	High Frequency Deafness
High Frequency Hearing Loss	High-Frequency Hearing Loss
Perceptive Deafness	Perceptive Hearing Loss
Perceptive Hearing Loss Or Deafness	Hearing Loss Sensorineural
Deafness Sensorineural	Hearing Loss High-Frequency
Hearing Loss, Central	Hearing Loss, High-Frequency

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 (14)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-N2360	Hinokiflavone	Inhibitor	99.80%	Unkown
HY-111532	(3R,4R)-A2-32-01	Inhibitor	99.73%	Unkown
HY-N1181	Tamarixetin	Inhibitor	99.31%	Unkown
HY-111532B	(3S,4S)-A2-32-01	Inhibitor	98.15%	Unkown
HY-164064	Anticancer agent 230	Activator	99.97%	Unkown
HY-152097	(S)-ZG197	Inhibitor	/	Unkown
HY-152096	(R)-ZG197	Inhibitor	/	Unkown
HY-163319	NCA029	Activator	/	Unkown
HY-P4284	Z-GGF-CMK		/	Unkown
HY-168062	ZYZ-17	Activator	/	Unkown
HY-155556	ZG36	Inhibitor	/	Unkown
HY-162650	SL44	Inhibitor	/	Unkown
HY-P4399	Z-Gly-Gly-Leu-AMC		/	Unkown
HY-126046	ACP1b	Activator	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Canis familiaris	CLPP	VGNC	VGNC:39356
Felis catus	CLPP	VGNC	VGNC:60973
Bos taurus	CLPP	VGNC	VGNC:27459
Macaca mulatta	CLPP	VGNC	VGNC:71263
Mus musculus	CLPP	MGD	MGI:1858213
Rattus norvegicus	CLPP	RGD	RGD:1588583

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