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Human Ornithine Aminotransferase Recombinant Protein (RPPB2059)

SKU:
RPPB2059
Product Type:
Recombinant Protein
Species:
Human
Uniprot:
P04181
Research Area:
Enzymes
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Description

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Product Name:Human Ornithine Aminotransferase Recombinant Protein
Product Code:RPPB2059
Size:25µg
Species:Human
Target:Ornithine Aminotransferase
Synonyms:DKFZp781A11155, HOGA, OATASE, Ornithine aminotransferase mitochondrial, Ornithine--oxo-acid aminotransferase, OAT, OKT, GACR.
Source:Escherichia Coli
Physical Appearance:Sterile filtered colorless solution.
Formulation:The Ornithine Aminotransferase protein solution contains 20mM Tris, pH-8, and 10% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MTVQGPPTSD DIFEREYKYG AHNYHPLPVA LERGKGIYLW DVEGRKYFDF LSSYSAVNQG HCHPKIVNAL KSQVDKLTLT SRAFYNNVLG EYEEYITKLF NYHKVLPMNT GVEAGETACK LARKWGYTVK GIQKYKAKIV AAGNFWGRT LSAISSSTDP TSYDGFGPFM PGFDIIPYND LPALERALQD PNVAAFMVEP IQGEAGVVVP DPGYLMGVRE LCTRHQVLFI ADEIQTGLAR TGRWLAVDYE NVRPDIVLLG KALSGGLYPV SAVLCDDDIM LTIKPGEHGS TYGGNPLGCR VAIAALEVLE EENLAENADK LGIILRNELM KLPSDVVTAV RGKGLLNAIV IKETKDWDAW KVCLRLRDNG LLAKPTHGDI IRFAPPLVIK EDELRESIEI INKTILSF

Ornithine Aminotransferase is a mitochondrial enzyme which is an important factor that converts arginine and ornithine into the major excitatory and inhibitory neurotransmitters glutamate and GABA. Ornithine Aminotransferase mutations result in a deficiency that cause the autosomal recessive eye disease Gyrate Atrophy.

Ornithine Aminotransferase Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 408 amino acids (33-439 a.a.) and having a molecular wieght of 45.2kDa.The Ornithine Aminotransferase is purified by proprietary chromatographic techniques.

UniProt Protein Function:OAT: Defects in OAT are the cause of hyperornithinemia with gyrate atrophy of choroid and retina (HOGA). HOGA is a slowly progressive blinding autosomal recessive disorder. Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Amino Acid Metabolism - arginine and proline; EC 2.6.1.13; Transferase; Mitochondrial

Chromosomal Location of Human Ortholog: 10q26

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function:identical protein binding; ornithine-oxo-acid transaminase activity; pyridoxal phosphate binding

Biological Process: amino acid biosynthetic process; arginine catabolic process to glutamate; arginine catabolic process to proline via ornithine; visual perception

Disease: Gyrate Atrophy Of Choroid And Retina

NCBI Summary:This gene encodes the mitochondrial enzyme ornithine aminotransferase, which is a key enzyme in the pathway that converts arginine and ornithine into the major excitatory and inhibitory neurotransmitters glutamate and GABA. Mutations that result in a deficiency of this enzyme cause the autosomal recessive eye disease Gyrate Atrophy. Alternatively spliced transcript variants encoding different isoforms have been described. Related pseudogenes have been defined on the X chromosome. [provided by RefSeq, Jan 2010]
UniProt Code:P04181
NCBI GenInfo Identifier:129018
NCBI Gene ID:4942
NCBI Accession:P04181.1
UniProt Secondary Accession:P04181,Q16068, Q16069, Q68CS0, Q6IAV9, Q9UD03, D3DRF0
UniProt Related Accession:P04181
Molecular Weight:32,853 Da
NCBI Full Name:Ornithine aminotransferase, mitochondrial
NCBI Synonym Full Names:ornithine aminotransferase
NCBI Official Symbol:OAT��
NCBI Official Synonym Symbols:OKT; GACR; HOGA; OATASE��
NCBI Protein Information:ornithine aminotransferase, mitochondrial
UniProt Protein Name:Ornithine aminotransferase, mitochondrial
UniProt Synonym Protein Names:Ornithine delta-aminotransferase; Ornithine--oxo-acid aminotransferase
UniProt Gene Name:OAT��
UniProt Entry Name:OAT_HUMAN

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