Peroxin

	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 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 w ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	PEX7 Peroxin-7 is a receptor associated with Refsum's disease and rhizomelic chondrodysplasia punctata Rhizomelic chondrodysplasia punctata is a rare developmental brain disorder characterized by systemic shortening of the proximal bones (i.e. rhizomelia), seizures, recurrent respiratory tract infections and congenital cataracts. The affected in ... type 1. See also * Peroxin External links GeneReviews/NCBI/NIH/UW entry on Refsum Disease GeneReviews/NIH/NCBI/UW entry on Rhizomelic Chondrodysplasia Punctata Type 1 * {{biochem-stub ... [...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 Peroxisome biogenesis factor 10 is a protein that in humans is encoded by the ''PEX10'' gene. Alternative splicing results in two t ... [...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 Adrenoleukodystrophy (ALD) is a disease linked to the X chromosome. It is a result of fatty acid buildup caused by peroxisomal fatty acid beta oxidation which results in the accumulation of very long chain fatty acids in tissues throughout the b ... to Zellweger syndrome. Interactions PEX10 has been shown to interact with PEX12 and PEX19. References Further reading * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis D ... [...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 In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a b .... Interactions PEX26 has been shown to interact with PEX1, PEX6 and SUFU. 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]
	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, * PEX13, * 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]
	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 In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''gener .... References Further reading * * * * * * * * * * * * * * * * External links ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	PEX13 Peroxisomal membrane protein PEX13 is a protein that in humans is encoded by the ''PEX13'' gene In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a b .... It located on chromosome 2 next to KIAA1841 Interactions PEX13 has been shown to interact with PEX14, PEX5 and PEX19. 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-2-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	PEX11G PEX11G is a human gene that encodes the peroxisomal biogenesis factor 11 gamma for peroxisomes. It is located on chromosome 19 Chromosome 19 is one of the 23 pairs of chromosomes in humans. People normally have two copies of this chromosome. Chromosome 19 spans more than 58.6 million base pairs, the building material of DNA. It is considered the most gene-rich chromosom .... References Genes on human chromosome 19 {{Gene-19-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]
	PEX11A Peroxisomal membrane protein 11A is a protein that in humans is encoded by the ''PEX11A'' gene In biology, the word gene (from , ; "... Wilhelm Johannsen coined the word gene to describe the Mendelian units of heredity..." meaning ''generation'' or ''birth'' or ''gender'') can have several different meanings. The Mendelian gene is a b .... References Further reading * * * * * * * * * {{gene-15-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 t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu]