2. Gene
  3. PEX19 - peroxisomal biogenesis factor 19 Gene

PEX19 - peroxisomal biogenesis factor 19 Gene

  • Gene
  • Protein
  • Disease
  • Agent
  • Ortholog
Homo sapiens

Also known as PXF; HK33; PMP1; PMPI; PXMP1; PBD12A; D1S2223E

Gene ID: 5824 | Gene type: protein coding

About PEX19

Cytogenetic location: 1q23.2 Genomic coordinates (GRCh38): 1:160,276,807-160,285,151 (from NCBI)

This gene has 12 transcripts (splice variants), 197 orthologues and is associated with 6 phenotypes. Ubiquitous expression in fat (RPKM 51.2), thyroid (RPKM 26.4) and 24 other tissues.

Summary

This gene is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2010]

PEX19 Products(2)

mRNA Protein Name
NM_001193644.1 NP_001180573.1 peroxisomal biogenesis factor 19 isoform c
NM_002857.4 NP_002848.1 peroxisomal biogenesis factor 19 isoform a

PEX19 Protein Structure

Pex19

Pex19: Pex19 protein family (57 - 299)

  • 0
  • 100
  • 200
  • 299 a.a.
Protein Preferred Names Protein Names

peroxisomal biogenesis factor 19

33 kDa housekeeping protein

PEX19 Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
PEX19 P40855 PSORS1C2 Homo sapiens Q9UIG4
Validated Y2H
32296183
Intra
PEX19 P40855 PXMP2 Homo sapiens Q9NR77
Pull Down
21102411
Intra
PEX19 P40855 PXMP2 Homo sapiens Q9NR77
Anti Tag CoIP
33961781
Intra
PEX19 P40855 PXMP2 Homo sapiens Q9NR77
Anti Tag CoIP
28514442
Intra
PEX19 P40855 NAT8L Homo sapiens Q8N9F0
Validated Y2H
32296183
Intra
PEX19 P40855 IL23A Homo sapiens Q9NPF7
Y2H Prey Pooling
25416956
Intra
PEX19 P40855 IL23A Homo sapiens Q9NPF7
Validated Y2H
32296183
Intra
PEX19 P40855 IL23A Homo sapiens Q9NPF7
Y2H Array
25416956
Intra
PEX19 P40855 TXN2 Homo sapiens Q99757
Validated Y2H
32296183
Intra
PEX19 P40855 PPIB Homo sapiens P23284
Y2H Array
25416956
Intra
PEX19 P40855 FKBP7 Homo sapiens Q9Y680
Validated Y2H
25416956
Intra
PEX19 P40855 FKBP7 Homo sapiens Q9Y680
Y2H Prey Pooling
25416956
Intra
PEX19 P40855 SUOX Homo sapiens P51687
Validated Y2H
32296183
Intra
PEX19 P40855 PEX11B Homo sapiens O96011
SLC
31467278
Intra
PEX19 P40855 PEX11B Homo sapiens O96011
Complementation
31467278
Intra
PEX19 P40855 PEX11B Homo sapiens O96011
Peptide Array
20531392
Intra
PEX19 P40855 PEX11B Homo sapiens O96011
Ub Reconstruction
12096124
Intra
PEX19 P40855 PEX11B Homo sapiens O96011
FPS
20531392
Intra
PEX19 P40855 PEX11B Homo sapiens O96011
BRET
29997244
Intra
PEX19 P40855 PEX12 Homo sapiens O00623
Y2H
10704444
Intra
PEX19 P40855 PEX13 Homo sapiens Q92968
Y2H
10704444
Intra
PEX19 P40855 PEX13 Homo sapiens Q92968
Far-WB
10704444
Intra
PEX19 P40855 PEX13 Homo sapiens Q92968
FPS
20531392
Intra
PEX19 P40855 PEX13 Homo sapiens Q92968
Peptide Array
20531392
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Validated Y2H
27107012
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Y2H Prey Pooling
25416956
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Y2H Array
31467278
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
SPR
21102411
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Far-WB
10704444
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Anti Tag CoIP
28514442
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
X-Ray Diffraction
21102411
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Y2H Array
25416956
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Y2H Array
25502805
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
BRET
29997244
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
SLC
31467278
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Y2H Pooling
16189514
Intra
PEX19 P40855 PEX3 Homo sapiens P56589
Pull Down
21102411
Intra
PEX19 P40855 SLC25A17 Homo sapiens O43808
Anti Tag CoIP
11402059
Intra
PEX19 P40855 PEX2 Homo sapiens P28328
Peptide Array
20531392
Intra
PEX19 P40855 PEX2 Homo sapiens P28328
Anti Tag CoIP
33961781
Intra
PEX19 P40855 PEX26 Homo sapiens Q7Z412
Validated Y2H
32296183
Intra
PEX19 P40855 PEX26 Homo sapiens Q7Z412
Peptide Array
20531392
Intra
PEX19 P40855 ABCD3 Homo sapiens P28288
IF
21102411
Intra
PEX19 P40855 ABCD3 Homo sapiens P28288
Anti Tag CoIP
14709540
Intra
PEX19 P40855 ABCD1 Homo sapiens P33897
Peptide Array
20531392
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
Lumier
32814053
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
Anti Tag CoIP
33961781
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
Peptide Array
20531392
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
Complementation
31467278
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
Ub Reconstruction
12096124
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
BRET
29997244
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
SLC
31467278
Intra
PEX19 P40855 PEX16 Homo sapiens Q9Y5Y5
MAPPIT
31467278
Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant PEX19 Proteins

Cat. No. Product Name Accession Purity
HY-P700383 PEX19 Protein, Human (GST) P40855 (A2-C296) ≥95%

Related Diseases

Diseases Alias
Peroxisome Biogenesis Disorder 12a

PBD12A

Peroxisome Biogenesis Disorder Complementation Group 14

PBD-CG14

Cg14

Pbd-Cgj

Peroxisome Biogenesis Disorder Complementation Group J

Peroxisome Biogenesis Disorder, Type 12a

Peroxisome Biogenesis Disorder, Complementation Group 14
Zellweger Syndrome

Cerebrohepatorenal Syndrome

Zellweger Leukodystrophy

Zs

Congenital Iron Overload

Chr

Zws

Severe Pbd-Zsd

Severe Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder
Neonatal Adrenoleukodystrophy

Nald

Adrenoleukodystrophy Autosomal Neonatal Form

Intermediate Pbd-Zsd

Intermediate Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

Adrenoleukodystrophy, Autosomal, Neonatal Form

Adrenoleukodystrophy Neonatal

Adrenoleukodystrophy, Neonatal
Peroxisome Biogenesis Disorder 1b

Peroxisome Biogenesis Disorder

Infantile Refsum Disease

Infantile Phytanic Acid Storage Disease

PBD1B

Refsum Disease, Infantile

Adrenoleukodystrophy, Autosomal Neonatal

Ird

Mild Pbd-Zsd

Mild Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

Pbd-Zsd

Peroxisome Biogenesis Disorder Spectrum

Peroxisome Biogenesis Disorder-Zellweger Spectrum Disorder

Autosomal Neonatal Adrenoleukodystrophy

Refsum Disease Infantile

Peroxisome Biogenesis Disorders

Peroxisome Biogenesis Disorder, Type 1b
Adrenoleukodystrophy

X-Linked Adrenoleukodystrophy

ALD

Siemerling-Creutzfeldt Disease

X-Ald

X-Linked Cerebral Adrenoleukodystrophy

Bronze Schilder Disease

Melanodermic Leukodystrophy

Addison Disease And Cerebral Sclerosis

Adrenomyeloneuropathy, Adult

Diffuse Sclerosis

X-Cald

Adrenomyeloneuropathy

Encephalitis Periaxialis Concentrica

Encephalitis Periaxialis, Schilder'S

Sudanophilic Cerebral Sclerosis

Ald Childhood Cerebral Form

Adrenoleukodystrophy X-Linked Cerebral Form

Adrenoleukodystrophy Childhood Cerebral Form

Childhood Cerebral Ald

Schilder Disease

X-Linked Ald

Adrenoleukodystrophy, X-Linked

Amn

Diffuse Cerebral Sclerosis Of Schilder

Systemic Scleroderma

Balo'S Concentric Sclerosis

Ald - [Adrenoleukodystrophy]

Addison-Schilder
Rhizomelic Chondrodysplasia Punctata

Chondrodysplasia Punctata, Rhizomelic

Rcdp

Chondrodysplasia Punctata, Rhizomelic Form

Rcp

Chondrodysplasia Punctata Rhizomelic
Encephalitozoonosis

Infection By Encephalitozoon
Peroxisomal Biogenesis Disorder

Zellweger Spectrum Disorders

Peroxisome Biogenesis Disorder-Zellweger Syndrome Spectrum

Disorders Of Peroxisome Biogenesis

Zellweger Spectrum

Zellweger Syndrome Spectrum

Peroxisomal Biogenesis Disorders

Pbd, Zss

Pbd-Zsd

Pbd-Zss

Pbd-Zellweger Spectrum Disorder

Peroxisomal Biogenesis Disorders, Zellweger Syndrome Spectrum

Peroxisome Biogenesis Disorder

Peroxisome Biogenesis Disorder Spectrum

Peroxisome Biogenesis Disorders

Zellweger Spectrum Disorder

Hyperpipecolic Acidaemia
Appendiceal Neoplasm

Appendix Neoplasm

Neoplasm Of Appendix

Appendiceal Neoplasms

Appendiceal Cancer

Appendix Cancer

Malignant Neoplasm Of Appendix Vermiformis

Malignant Tumour Of Appendix
Coccidioidomycosis

Valley Fever

Primary Extrapulmonary Coccidioidomycosis

Coccidioides Immitis Infection

Coccidiosis

Coccidioides Infection

Coccidioidomycosis, Unspecified

San Joaquin Fever

California Disease

Desert Fever

Desert Rheumatism

San Joaquin Valley Fever

Enteric Coccidiosis

Primary Pulmonary Coccidioidomycosis

Acute Coccidioidomycosis

Posadas Wernicke Disease
Appendix Cancer

Appendiceal Neoplasms

Cancer Of The Appendix

Malignant Neoplasm Of Appendix Vermiformis

Malignant Tumor Of Appendix

Malignant Tumor Of The Appendix

Malignant Neoplasm Of Appendix

Carcinoma Of The Appendix
Appendix Disease

Disorder Of Appendix
Peroxisomal Disease

Peroxisomal Disorder

Peroxisomal Disorders

Peroxisomal Defects
Acatalasemia

Acatalasia

Catalase Deficiency

Deficiency Of Catalase

ACATLAS

Takahara'S Disease

Takahara Disease
Chondrodysplasia Punctata Syndrome

Chondrodysplasia Punctata

Chondrodysplasia Punctata Congenita

Toriello Higgins Miller Syndrome

Chondrodysplasia Punctata, Toriello Type

Toriello-Higgins-Miller Syndrome

Cdp

Chondrodysplasia Punctata, X-Linked Dominant Type

Chondrodysplasia Punctata Group

Dysplasia Punctata Epiphysis

Dysplasia Punctata

Dysplasia Epiphysealis Punctata

Chondrodystrophy Of Punctata
Methylmalonic Aciduria And Homocystinuria, Cbld Type

Homocystinuria, Cbld Type, Variant 1

Methylmalonic Aciduria And Homocystinuria Type Cbld

Vitamin B12-Responsive Methylmalonic Acidemia, Type Cbldv2

MAHCD

Methylmalonic Acidemia And Homocystinuria, Cbld Type

Methylmalonic Aciduria, Cblh Type, Formerly

Methylmalonic Acidemia, Cblh Type, Formerly

Methylmalonic Aciduria, Cbld Type, Variant 2

Cobalamin D Deficiency

Methylcobalamin Deficiency Type Cbldv1

Functional Methionine Synthase Deficiency Type Cbldv1

Vitamin B12-Responsive Methylmalonic Aciduria, Type Cbldv2

Methylmalonic Acidemia With Homocystinuria, Type Cbld

Cbld Defect

Cobalamin D Defect

Combined Defect In Adenosylcobalamin And Methylcobalamin Synthesis, Type Cbld

Methylmalonic Aciduria With Homocystinuria, Type Cbld

Homocystinuria Cbld Variant 1

Methylmalonic Acidemia And Homocystinuria Cbld Type

Methylmalonic Aciduria And Homocystinuria Cbld-Combined

Methylmalonic Aciduria And Homocystinuria Cbld Original

Methylmalonic Aciduria Cbld Variant 2

Aciduria, Methylmalonic, And Homocystinuria, Cbld Type
Microsporidiosis

Infection By Microspora

Microsporidiasis

Infection By Microsporea

Infection By Microsporida

Intestinal Microsporidiosis

Microsporidia Infection

Infection By Microsporidia
Refsum Disease, Classic

Refsum Disease

Heredopathia Atactica Polyneuritiformis

Phytanic Acid Oxidase Deficiency

Hmsn Iv

Refsum Disease, Adult, 1

Refsum'S Disease

Phytanic Acid Storage Disease

Hereditary Motor And Sensory Neuropathy Iv

Hmsn4

Hmsn Type Iv

Hmsn 4

Adult Refsum Disease

Classic Refsum Disease

Hereditary Motor And Sensory Neuropathy Type Iv

Refsum Syndrome

Hsmn Iv

Disorder Of Cornification 11

Doc 11

Hereditary Sensory And Motor Neuropathy Type 4

Hypertrophic Neuropathy Of Refsum

Ard

Crd

Hereditary Motor And Sensory Neuropathy Type 4

Phytanic-Coa Hydroxylase Deficiency

RD
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-RS10347 PEX19 Human Pre-designed siRNA Set A Pre-designed Sets / Unkown

Orthologs Information

Species Symbol Source ID
Rattus norvegicus PEX19 RGD RGD:1306913
Bos taurus PEX19 VGNC VGNC:32758
Canis familiaris PEX19 VGNC VGNC:44432
Mus musculus PEX19 MGD MGI:1334458
Felis catus PEX19 VGNC VGNC:69189
Others PEX19 NCBI