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Peroxin
Peroxins (or peroxisomal/peroxisome biogenesis factors) represent several protein families found in peroxisomes. Deficiencies are associated with several peroxisomal disorders. Peroxins serve several functions including the recognition of cytoplasmic proteins that contain peroxisomal targeting signals (PTS) that tag them for transport by peroxisomal proteins to the peroxisome. Peroxins are structurally diverse and have been classified to different protein families. Some of them were predicted to be single-pass transmembrane proteins, for example Peroxisomal biogenesis factor 11 Pernoxin is a value of venomosity to animalia. Genes * PEX1 * PEX2 * PEX3 * PEX5 * PEX6 * PEX7 * PEX10 * PEX11A, PEX11B, PEX11G * PEX12 * PEX13 * PEX14 * PEX16 * PEX19 * PEX26 References Gene families Transmembrane proteins {{biochem-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Peroxisome
A peroxisome () is a membrane-bound organelle, a type of microbody, found in the cytoplasm of virtually all eukaryotic cells. Peroxisomes are oxidative organelles. Frequently, molecular oxygen serves as a co-substrate, from which hydrogen peroxide (H2O2) is then formed. Peroxisomes owe their name to hydrogen peroxide generating and scavenging activities. They perform key roles in lipid metabolism and the conversion of reactive oxygen species. Peroxisomes are involved in the catabolism of very long chain fatty acids, branched chain fatty acids, bile acid intermediates (in the liver), D-amino acids, and polyamines, the reduction of reactive oxygen species – specifically hydrogen peroxide – and the biosynthesis of plasmalogens, i.e., ether phospholipids critical for the normal function of mammalian brains and lungs. They also contain approximately 10% of the total activity of two enzymes (Glucose-6-phosphate dehydrogenase and 6-Phosphogluconate dehydrogenase) in the pentose ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX12
Peroxisome assembly protein 12 is a protein that in humans is encoded by the ''PEX12'' gene. Function PEX12 is needed for protein import into peroxisomes. This gene belongs to the peroxin-12 family. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. Clinical significance The peroxisome biogenesis disorders (PBDs; MIM 601539) are a group of genetically heterogeneous diseases that are usually lethal in early infancy. 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. This cellular phenotype is shared by yeast 'pex' mutants, and human orthologs of yeast PEX genes defective in some PBD complementation groups (CGs). Interactions PEX12 has been shown to interact with PEX10, PEX5 and PEX19 Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the ''PEX19'' gene. Interactions PEX19 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX6
Peroxisome assembly factor 2 is a protein that in humans is encoded by the ''PEX6'' gene. PEX6 is an AAA ATPase that localizes to the peroxisome. PEX6 forms a hexamer with PEX1 and is recruited to the membrane by PEX26. Function From yeast to plants to humans, there is only one verified function of PEX6; PEX6 (and PEX1) removes PEX5 from the peroxisomal membrane so that PEX5 may do additional rounds of peroxisomal import. Human PEX6 can genetically complement plant ''pex6'' mutants, which highlights functional conservation. Work with ''pex6'' mutants in '' Arabidopsis thaliana'' has shown that PEX6 may have a role in consuming oil body (plant-specific lipid droplets). Work with yeast ''pex6'' mutants has shown that PEX6 is a key player in the autophagy of peroxisomes called pexophagy. Related diseases Mutations in the genes encoding PEX6, along with PEX1, are the leading causes of peroxisomal biogenesis disorders, such as Zellweger Syndrome spectrum, infantile Refsum dise ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX14
Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the ''PEX14'' gene. Function This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome. Interactions PEX14 has been shown to interact with * PEX5, * PEX7, and * PEX13 Peroxisomal membrane protein PEX13 is a protein that in humans is encoded by the ''PEX13'' gene. It located on chromosome 2 next to KIAA1841 Interactions PEX13 has been shown to interact with PEX14, PEX5 and PEX19 Peroxisomal biogenesis fact .... References Further reading * * * * * * * * * * * * * * * * External links ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX5
Peroxisomal targeting signal 1 receptor (PTS1R) is a protein that in humans is encoded by the ''PEX5'' gene. PTS1R is a peroxisomal targeting sequence involved in the specific transport of molecules for oxidation inside the peroxisome. SKL binds to PTS1R in the cytosol followed by binding to the Pex14p receptor allowing importation of the peroxisomal protein through the pexsubunit transporter. Diseases associated with dysfunctional PTS1R receptors include X-linked adrenoleukodystrophy and Zellweger syndrome. Interactions PEX5 has been shown to interact with PEX12, PEX13 and PEX14 Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the ''PEX14'' gene. Function This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with i .... References Further reading * * * * * * * * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX1
Peroxisome biogenesis factor 1, also known as PEX1, is a protein which in humans is encoded by the ''PEX1'' gene. This gene encodes a member of the AAA protein family, a large group of ATPases associated with diverse cellular activities. This protein is cytoplasmic but is often anchored to a peroxisomal membrane where it forms a heteromeric complex and plays a role in the import of proteins into peroxisomes and peroxisome biogenesis. Mutations in this gene have been associated with complementation group 1 peroxisomal disorders such as neonatal adrenoleukodystrophy, infantile Refsum disease, and Zellweger syndrome. Interactions PEX1 has been shown to interact with PEX6 and PEX26 Peroxisome assembly protein 26 is a protein that in humans is encoded by the ''PEX26'' gene. Interactions PEX26 has been shown to interact with PEX1, PEX6 and SUFU Suppressor of fused homolog is a protein that in humans is encoded by the ' .... References Further reading * * * * * * * * * * ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX10
Peroxisome biogenesis factor 10 is a protein that in humans is encoded by the ''PEX10'' gene. Alternative splicing results in two transcript variants encoding different isoforms. Function Peroxisome biogenesis factor 10 is involved in import of peroxisomal matrix proteins. This protein localizes to the peroxisomal membrane. Clinical significance Mutations in this gene result in phenotypes within the Zellweger spectrum of peroxisomal biogenesis disorders, ranging from neonatal adrenoleukodystrophy to Zellweger syndrome. Interactions PEX10 has been shown to interact with PEX12 and PEX19 Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the ''PEX19'' gene. Interactions PEX19 has been shown to Protein-protein interaction, interact with: * ABCD1, * ABCD2, * ABCD3, * PEX10, * PEX11B, * PEX12, * PEX .... References Further reading * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis Disord ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX26
Peroxisome assembly protein 26 is a protein that in humans is encoded by the ''PEX26'' gene. Interactions PEX26 has been shown to interact with PEX1, PEX6 and SUFU Suppressor of fused homolog is a protein that in humans is encoded by the ''SUFU'' gene. In molecular biology, the protein domain suppressor of fused protein (Sufu) has an important role in the cell. The Sufu is important in negatively regulating .... References Further reading * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis Disorders, Zellweger Syndrome SpectrumOMIM entries on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum {{protein-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX3
Peroxisomal biogenesis factor 3 is a protein that in humans is encoded by the ''PEX3'' gene. Interactions PEX3 has been shown to interact with PEX19 Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the ''PEX19'' gene. Interactions PEX19 has been shown to interact with: * ABCD1, * ABCD2, * ABCD3, * PEX10, * PEX11B, * PEX12, * PEX13 Peroxisomal membrane p .... References Further reading * * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis Disorders, Zellweger Syndrome SpectrumOMIM entries on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum {{gene-6-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX2
Peroxisomal biogenesis factor 2 is a protein that in humans is encoded by the ''PEX2'' gene. This gene encodes an integral peroxisomal membrane protein required for peroxisome biogenesis. The protein is thought to be involved in peroxisomal matrix protein import. Mutations in this gene result in one form of Zellweger syndrome and infantile Refsum disease Infantile Refsum disease (IRD) is a rare autosomal recessive congenital peroxisomal biogenesis disorder within the Zellweger spectrum. These are disorders of the peroxisomes that are clinically similar to Zellweger syndrome and associated with .... Alternative splicing results in multiple transcript variants encoding the same protein. References Further reading * * * * * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis Disorders, Zellweger Syndrome SpectrumOMIM entries on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum {{gene-8-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
PEX19
Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the ''PEX19'' gene. Interactions PEX19 has been shown to interact with: * ABCD1, * ABCD2, * ABCD3, * PEX10, * PEX11B, * PEX12, * PEX13 Peroxisomal membrane protein PEX13 is a protein that in humans is encoded by the ''PEX13'' gene. It located on chromosome 2 next to KIAA1841 Interactions PEX13 has been shown to interact with PEX14, PEX5 and PEX19 Peroxisomal biogenesis fact ..., * PEX16, and * PEX3. References Further reading * * * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis Disorders, Zellweger Syndrome SpectrumOMIM entries on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum * {{PDBe-KB2, P40855, Peroxisomal biogenesis factor 19 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Peroxisomal Biogenesis Factor 11
Peroxisomal biogenesis factor 11 (PEX11) are peroxin, peroxisomal membrane proteins which promote peroxisome division in eukaryotic cells. Human proteins from this family * PEX11A * PEX11B * PEX11G References Protein domains Protein families Membrane proteins {{membrane-protein-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |