{ "uniprot_canonical_ac": "A2VCW9-1", "sequence": { "header": "sp|A2VCW9-1|AASS_RAT Isoform 1 of Alpha-aminoadipic semialdehyde synthase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Aass PE=2 SV=1 CANONICAL=A2VCW9-1", "length": 926, "sequence": "MLRAQRLRLARLRACVSRGLHHKPVMALRREDVNAWERRAPLAPKHIKGITKLGYKVLIQPSNRRAIHDKEYVRAGGILQEDITEACLILGVKRPPEEKLMSKKTYAFFSHTIKAQEANMSLLDEVLKQEIRLIDYEKMVDHRGSRIVAFGHWAGVAGMINILHGMGLRLLALGHHTPFMHLGMAHNYRNSSQAVQAVRDAGYEISLGLMPKSIGPLTFVFTGTGNVSKGAQEVFNELPCEYVEPHELKEVSKTGDLRKVYGTVLSRHHHLVRKTDGVYDPVEYEKYPERYISRFNADIAPYTTCLINGIYWEQNTPRLLTRQDAQSLLVPVKSSVVPVEGCPELPHKLVAICDISADTGGSIDFMTECTTIERPFCMYDADQHIIHDSVEGSGILMCSIDNLPAQLPIEATEYFGDMLYPYVEEMLLSDASQPLESQNFSPVVRDAVITSNGLLTDKYKYIQKLRESRERIQFLSMSTKKKVLVLGSGYVSGPVLEYLSRGNNIEITLGSDMTNQMQQLSKKYDINTVNVTVGKQEDKLQSLVESQDLVISLLPYVLHPVVAKACIDSKVNMVTASYITPAMKELEKSVDDAGITVIGELGLDPGLDHMLAMETIDKAKDLGATIESYVSYCGGLPAPEHSDNPLRYKFSWSPVGVLMNIMQPASYLLNGKVVNVTGGVSFLNSVTPMDYFPGLNLEGYPNRDSTKYAEIYGISSAHTLLRGTLRYKGYSKALNGFVKLGLINRETYPALRPEANPLTWKQLLCDLVGISRSSSCEKLKEVVFTKLGGDSTQLEAAEWLGLLGDEQVPQAESIVDAFSKHLVSKLSYGPEEKDMIVMRDSFGIRHPSGHLENKTIDLVVYGDFNGFSAMAKTVGLPTAMAAKMLLDGEIETKGLMGPFSKEIYGPILERIKAEGIVFNTQSTIKL" }, "uniprot_id": "AASS_RAT", "enzyme_annotation": [ { "ec": "1.5.1.8", "activity": "N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP(+) + H(2)O = L-lysine + 2-oxoglutarate + NADPH." }, { "ec": "1.5.1.9", "activity": "N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD(+) + H(2)O = L-glutamate + (S)-2-amino-6-oxohexanoate + NADH." } ], "synonyms": { "protein": { "refseq": [ "alpha-aminoadipic semialdehyde synthase, mitochondrial precursor", "alpha-aminoadipic semialdehyde synthase, mitochondrial; LKR/SDH" ], "uniprotkb": [ "Alpha-aminoadipic semialdehyde synthase, mitochondrial", "SDH" ] }, "gene": { "refseq": [ "Aass" ], "uniprotkb": [ "Aass" ] } }, "keywords": [], "species": [ { "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "Rattus norvegicus", "taxid": 10116 } ], "recommendedname": { "full": "Alpha-aminoadipic semialdehyde synthase, mitochondrial; SDH." }, "publication": [ { "title": "Lysine degradation through the saccharopine pathway in mammals: involvement of both bifunctional and monofunctional lysine-degrading enzymes in mouse.", "url": "https://www.ncbi.nlm.nih.gov/pubmed/10567240", "journal": "The Biochemical journal", "evidence": [ { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_001094433.1", "id": "NP_001094433.1", "database": "RefSeq" } ], "authors": "Papes F, Kemper EL, Cord-Neto G, Langone F, Arruda P", "date": "1999", "pmid": "10567240" }, { "title": "Familial hyperlysinemias. Purification and characterization of the bifunctional aminoadipic semialdehyde synthase with lysine-ketoglutarate reductase and saccharopine dehydrogenase activities.", "url": "https://www.ncbi.nlm.nih.gov/pubmed/6434529", "journal": "The Journal of biological chemistry", "evidence": [ { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_001094433.1", "id": "NP_001094433.1", "database": "RefSeq" } ], "authors": "Markovitz PJ, Chuang DT, Cox RP", "date": "1984", "pmid": "6434529" } ], "glycosylation": [], "mutation": [], "isoforms": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9#A2VCW9-1", "isoform_ac": "A2VCW9-1", "locus": { "end_pos": 0, "start_pos": 0, "chromosome": "N/A", "evidence": [] }, "sequence": { "header": "sp|A2VCW9-1|AASS_RAT Isoform 1 of Alpha-aminoadipic semialdehyde synthase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Aass PE=2 SV=1 CANONICAL=A2VCW9-1", "length": 926, "sequence": "MLRAQRLRLARLRACVSRGLHHKPVMALRREDVNAWERRAPLAPKHIKGITKLGYKVLIQPSNRRAIHDKEYVRAGGILQEDITEACLILGVKRPPEEKLMSKKTYAFFSHTIKAQEANMSLLDEVLKQEIRLIDYEKMVDHRGSRIVAFGHWAGVAGMINILHGMGLRLLALGHHTPFMHLGMAHNYRNSSQAVQAVRDAGYEISLGLMPKSIGPLTFVFTGTGNVSKGAQEVFNELPCEYVEPHELKEVSKTGDLRKVYGTVLSRHHHLVRKTDGVYDPVEYEKYPERYISRFNADIAPYTTCLINGIYWEQNTPRLLTRQDAQSLLVPVKSSVVPVEGCPELPHKLVAICDISADTGGSIDFMTECTTIERPFCMYDADQHIIHDSVEGSGILMCSIDNLPAQLPIEATEYFGDMLYPYVEEMLLSDASQPLESQNFSPVVRDAVITSNGLLTDKYKYIQKLRESRERIQFLSMSTKKKVLVLGSGYVSGPVLEYLSRGNNIEITLGSDMTNQMQQLSKKYDINTVNVTVGKQEDKLQSLVESQDLVISLLPYVLHPVVAKACIDSKVNMVTASYITPAMKELEKSVDDAGITVIGELGLDPGLDHMLAMETIDKAKDLGATIESYVSYCGGLPAPEHSDNPLRYKFSWSPVGVLMNIMQPASYLLNGKVVNVTGGVSFLNSVTPMDYFPGLNLEGYPNRDSTKYAEIYGISSAHTLLRGTLRYKGYSKALNGFVKLGLINRETYPALRPEANPLTWKQLLCDLVGISRSSSCEKLKEVVFTKLGGDSTQLEAAEWLGLLGDEQVPQAESIVDAFSKHLVSKLSYGPEEKDMIVMRDSFGIRHPSGHLENKTIDLVVYGDFNGFSAMAKTVGLPTAMAAKMLLDGEIETKGLMGPFSKEIYGPILERIKAEGIVFNTQSTIKL" } }, { "url": "https://www.uniprot.org/uniprot/A2VCW9#D4ACE9-1", "isoform_ac": "D4ACE9-1", "locus": { "end_pos": 50152455, "start_pos": 50200328, "chromosome": "4", "evidence": [ { "url": "https://www.ensembl.org/id/ENSRNOT00000060530", "id": "ENSRNOT00000060530", "database": "Ensembl Transcript" }, { "url": "https://www.ensembl.org/id/ENSRNOP00000057271", "id": "ENSRNOP00000057271", "database": "Ensembl Peptide" } ] }, "sequence": { "header": "tr|D4ACE9-1|AASS_RAT Computationally mapped isoform of Alpha-aminoadipic semialdehyde synthase, mitochondrial OS=Rattus norvegicus OX=10116 GN=Aass PE=2 SV=3 CANONICAL=A2VCW9-1", "length": 926, "sequence": "MLRAQRLRLARLRACVSRGLHHKPVMALRREDVNAWERRAPLAPKHIKGITKLGYKVLIQPSNRRAIHDKEYVRAGGILQEDITEACLILGVKRPPEEKLMSKKTYAFFSHTIKAQEANMSLLDEVLKQEIRLIDYEKMVDHRGSRIVAFGQWAGVAGMINILHGMGLRLLALGHHTPFMHLGMAHNYRNSSQAVQAVRDAGYEISLGLMPKSIGPLTFVFTGTGNVSKGAQEVFNELPCEYVEPHELKEVSKTGDLRKVYGTVLSRHHHLVRKTDGVYDPVEYEKYPERYISRFNADIAPYTTFRICGIGWHCGSRRISISIQDCYLIKVKIKLICRVDTCYVAVIFRLVAICDISADTGGSIDFMTECTTIERPFCMYDADQHIIHDSVEGSGILMCSIDNLPAQLPIEATEYFGDMLYPYVEEMLLSDASQPLESQNFSPVVRDAVITSNGLLTDKYKYIQKLRESRERIQFLSMSTKKKVLVLGSGYVSGPVLEYLSRGNNIEITLGSDMTNQMQQLSKKYDINTVNVTVGKQEDKLQSLVESQDLVISLLPYVLHPVVAKACIDSKVNMVTASYITPAMKELEKSVDDAGITVIGELGLDPGLDHMLAMETIDKAKDLGATIESYVSYCGGLPAPEHSDNPLRYKFSWSPVGVLMNIMQPASYLLNGKVVNVTGGVSFLNSVPMDYFPGLNLEGYPNRDSTKYAEIYGISSAHTLLRGTLRYKGYSKALNGFVKLGLINRETYPALRPEANPLTWKQLLCDLVGISRSSSCEKLKEVVFTKLGGDSTQLEAAEWLGLLGDEQVPQAESIVDAFSKHLVSKLSYGPEEKDMIVMRDSFGIRHPSGHLENKTIDLVVYGDFNGFSAMAKTVGLPTAMAAKMLLDGEIETKGLMGPFSKEIYGPILERIKAEGIVFNTQSTIKL" } } ], "pathway": [ { "url": "https://www.genome.jp/dbget-bin/www_bget?rno:296925", "resource": "KEGG Pathway", "id": "rno:296925", "name": "" }, { "url": "https://www.reactome.org/PathwayBrowser/#R-RNO-71064", "resource": "Reactome", "id": "R-RNO-71064", "name": "Lysine catabolism" } ], "function": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9#function", "annotation": "Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-oxoglutarate reductase and saccharopine dehydrogenase activity, respectively (By similarity).", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9#function", "id": "A2VCW9", "database": "UniProtKB" } ] } ], "orthologs": [ { "uniprot_canonical_ac": "Q9UDR5-1", "protein_name": "Alpha-aminoadipic semialdehyde synthase, mitochondrial; SDH.", "sequence": { "length": 926, "sequence": "MLQVHRTGLGRLGVSLSKGLHHKAVLAVRREDVNAWERRAPLAPKHIKGITNLGYKVLIQPSNRRAIHDKDYVKAGGILQEDISEACLILGVKRPPEEKLMSRKTYAFFSHTIKAQEANMGLLDEILKQEIRLIDYEKMVDHRGVRVVAFGQWAGVAGMINILHGMGLRLLALGHHTPFMHIGMAHNYRNSSQAVQAVRDAGYEISLGLMPKSIGPLTFVFTGTGNVSKGAQAIFNELPCEYVEPHELKEVSQTGDLRKVYGTVLSRHHHLVRKTDAVYDPAEYDKHPERYISRFNTDIAPYTTCLINGIYWEQNTPRLLTRQDAQSLLAPGKFSPAGVEGCPALPHKLVAICDISADTGGSIEFMTECTTIEHPFCMYDADQHIIHDSVEGSGILMCSIDNLPAQLPIEATECFGDMLYPYVEEMILSDATQPLESQNFSPVVRDAVITSNGTLPDKYKYIQTLRESRERAQSLSMGTRRKVLVLGSGYISEPVLEYLSRDGNIEITVGSDMKNQIEQLGKKYNINPVSMDICKQEEKLGFLVAKQDLVISLLPYVLHPLVAKACITNKVNMVTASYITPALKELEKSVEDAGITIIGELGLDPGLDHMLAMETIDKAKEVGATIESYISYCGGLPAPEHSNNPLRYKFSWSPVGVLMNVMQSATYLLDGKVVNVAGGISFLDAVTSMDFFPGLNLEGYPNRDSTKYAEIYGISSAHTLLRGTLRYKGYMKALNGFVKLGLINREALPAFRPEANPLTWKQLLCDLVGISPSSEHDVLKEAVLKKLGGDNTQLEAAEWLGLLGDEQVPQAESILDALSKHLVMKLSYGPEEKDMIVMRDSFGIRHPSGHLEHKTIDLVAYGDINGFSAMAKTVGLPTAMAAKMLLDGEIGAKGLMGPFSKEIYGPILERIKAEGIIYTTQSTIKP" }, "evidence": [ { "url": "http://www.informatics.jax.org/homology/4212", "id": "4212", "database": "MGI" } ], "organism": "Homo sapiens", "tax_id": 9606 }, { "uniprot_canonical_ac": "Q99K67-1", "protein_name": "Alpha-aminoadipic semialdehyde synthase, mitochondrial; SDH.", "sequence": { "length": 926, "sequence": "MLRAQRPRLARLRACLSRGLHHKPVMALRREDVNAWERRAPLAPKHIKGITKLGYKVLIQPSNRRAIHDKEYVRAGGILQEDITEACLILGVKRPPEEKLMSKKTYAFFSHTIKAQEANMNLLDEVLKQEIRLIDYEKMVDHRGSRIVAFGQWAGVAGMINILHGMGLRLLALGHHTPFMHLGMAHNYRNSSQAVQAVRDAGYEISLGLMPKSIGPLTFVFTGTGNVSKGAQEVFNELPCEYVEPHELREVSKTGDLRKVYGTVLSRHHHLVRKTDGVYDPVEYEKYPERYTSRFNTDIAPYTTCLINGIYWEQNTPRLLTRQDAQSLLVPVKSSVVPVEGCPELPHKLVAICDISADTGGSIDFMTECTTIERPFCMYDADQQIIHDSVEGSGILMCSIDNLPAQLPIEATEYFGDMLYPYVEEMLLSDASQPLESQNFSPVVRDAVITSNGLLTDKYKYIQKLRESRERIQFLSMSTKKKVLVLGSGYVSGPVLEYLSRDNNIEITLGSDMTNQMQQLSKKYNINPVSLTVGKQEAKLQSLVESQDLVISLLPYVLHPVVAKACIESRVNMVTASYITPAMKELEKSVDDAGITVIGELGLDPGLDHMLAMETIDTAKELGATVESYVSYCGGLPAPEHSDNPLRYKFSWSPVGVLMNIMQPASYLLNGKVVNVTGGVSFLNSVTPMDYFPGLNLEGYPNRDSIKYAEIYGISSAHTLLRGTLRYKGYSKALNGFVKLGLINREAYPALRPEANPLTWKQLLCDLVGISRSSPCEKLKEVVFTKLGGDNTQLEAAEWLGLLGDEQVPQAESIVDAFSKHLVSKLSYGPEEKDMIVMRDSFGIRHPSGHLENKTIDLVVYGDFNGFSAMAKTVGLPTAMAAKMLLDGEIEAKGLMGPFTKEIYGPILERIKAEGIVFNTQSTIKL" }, "evidence": [ { "url": "http://www.informatics.jax.org/homology/4212", "id": "4212", "database": "MGI" } ], "organism": "Mus musculus", "tax_id": 10090 } ], "refseq": { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_001094433.1", "ac": "NP_001094433.1", "name": "alpha-aminoadipic semialdehyde synthase, mitochondrial precursor; alpha-aminoadipic semialdehyde synthase, mitochondrial; LKR/SDH.", "summary": "" }, "go_annotation": { "categories": [ { "go_terms": [ { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0004753", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "saccharopine dehydrogenase activity", "id": "GO:0004753" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0047130", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "saccharopine dehydrogenase (NADP+, L-lysine-forming) activity", "id": "GO:0047130" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0047131", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity", "id": "GO:0047131" } ], "total": 3, "name": "Molecular Function" }, { "go_terms": [ { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005737", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "cytoplasm", "id": "GO:0005737" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0005739", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "mitochondrion", "id": "GO:0005739" } ], "total": 2, "name": "Cellular Component" }, { "go_terms": [ { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0019878", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "lysine biosynthetic process via aminoadipic acid", "id": "GO:0019878" }, { "url": "https://www.ebi.ac.uk/QuickGO/term/GO:0033512", "evidence": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "id": "A2VCW9", "database": "UniProtKB" } ], "name": "L-lysine catabolic process to acetyl-CoA via saccharopine", "id": "GO:0033512" } ], "total": 2, "name": "Biological Process" } ] }, "site_annotation": [ { "end_pos": 121, "annotation": "n_glycosylation_sequon", "start_pos": 119 }, { "end_pos": 192, "annotation": "n_glycosylation_sequon", "start_pos": 190 }, { "end_pos": 228, "annotation": "n_glycosylation_sequon", "start_pos": 226 }, { "end_pos": 441, "annotation": "n_glycosylation_sequon", "start_pos": 439 }, { "end_pos": 532, "annotation": "n_glycosylation_sequon", "start_pos": 530 }, { "end_pos": 677, "annotation": "n_glycosylation_sequon", "start_pos": 675 }, { "end_pos": 855, "annotation": "n_glycosylation_sequon", "start_pos": 853 } ], "crossref": [ { "url": "https://www.ncbi.nlm.nih.gov/protein/NP_001094433.1", "id": "NP_001094433.1", "database": "RefSeq" }, { "url": "https://www.reactome.org/PathwayBrowser/#R-RNO-71064", "id": "R-RNO-71064", "database": "Reactome" }, { "url": "https://www.genome.jp/dbget-bin/www_bget?rno:296925", "id": "rno:296925", "database": "KEGG Pathway" }, { "url": "https://www.ebi.ac.uk/interpro/entry/IPR005097", "id": "IPR005097", "database": "InterPro" }, { "url": "https://www.ebi.ac.uk/interpro/entry/IPR007698", "id": "IPR007698", "database": "InterPro" }, { "url": "https://www.ebi.ac.uk/interpro/entry/IPR007886", "id": "IPR007886", "database": "InterPro" }, { "url": "https://www.ebi.ac.uk/interpro/entry/IPR032095", "id": "IPR032095", "database": "InterPro" }, { "url": "https://www.ebi.ac.uk/interpro/entry/IPR036291", "id": "IPR036291", "database": "InterPro" }, { "url": "http://pfam.xfam.org/family/PF03435", "id": "PF03435", "database": "Pfam" }, { "url": "http://pfam.xfam.org/family/PF05222", "id": "PF05222", "database": "Pfam" }, { "url": "http://pfam.xfam.org/family/PF16653", "id": "PF16653", "database": "Pfam" }, { "url": "http://research.bioinformatics.udel.edu/pro/entry/PR_A2VCW9", "id": "PR_A2VCW9", "database": "PRO" }, { "url": "https://www.ncbi.nlm.nih.gov/gene/296925", "id": "296925", "database": "GeneID" }, { "url": "http://rgd.mcw.edu/tools/genes/genes_view.cgi?id=1310811", "id": "1310811", "database": "RGD" } ], "expression_disease": [], "uniprot_ac": "A2VCW9", "disease": [], "mass": { "monoisotopic_mass": 102908.0, "chemical_mass": 102908.0 }, "expression_tissue": [], "gene": [ { "url": "https://www.uniprot.org/uniprot/A2VCW9", "name": "Aass.", "locus": { "end_pos": 50152452, "start_pos": 50200328, "chromosome": "4", "evidence": [ { "url": "https://www.ensembl.org/id/ENSRNOG00000039494", "id": "ENSRNOG00000039494", "database": "Ensembl Gene" } ] } } ] }