Showing Protein Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial (HMDBP00712)

• 5985

1. 3-oxoacid-CoA transferase 1
2. SCOT-s
3. Somatic-type succinyl-CoA:3-oxoacid-CoA-transferase
4. 3-oxoacid CoA-transferase 1
5. Somatic-type succinyl-CoA:3-oxoacid CoA-transferase

• 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency
• 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
• 3-hydroxyisobutyric acid dehydrogenase deficiency
• 3-hydroxyisobutyric aciduria
• 3-Methylcrotonyl Coa Carboxylase Deficiency Type I
• 3-Methylglutaconic Aciduria Type I
• 3-Methylglutaconic Aciduria Type III
• 3-Methylglutaconic Aciduria Type IV
• Beta-Ketothiolase Deficiency
• Butanoate metabolism
• Butyrate Metabolism
• Isobutyryl-coa dehydrogenase deficiency
• Isovaleric acidemia
• Isovaleric Aciduria
• Ketone Body Metabolism
• Maple Syrup Urine Disease
• Methylmalonate Semialdehyde Dehydrogenase Deficiency
• Methylmalonic Aciduria
• Propionic Acidemia
• Succinyl CoA: 3-ketoacid CoA transferase deficiency
• succinyl-CoA degradation
• Synthesis and degradation of ketone bodies
• Valine, leucine and isoleucine degradation
• Valine, leucine and isoleucine degradation

1. Mitochondrion matrix

>1563 bp
ATGGCGGCTCTCAAACTCCTCTCCTCCGGGCTTCGGCTCTGCGCCTCTGCCCGCGGATCT
GGGGCAACCTGGTACAAGGGATGTGTTTGTTCCTTTTCCACCAGTGCTCATCGCCATACC
AAGTTTTATACAGATCCAGTAGAAGCTGTAAAAGACATCCCTGATGGTGCCACGGTTTTG
GTTGGTGGTTTTGGGCTATGTGGAATTCCAGAGAATCTTATAGATGCTTTACTGAAAACT
GGAGTAAAAGGACTAACTGCAGTCAGCAACAATGCAGGGGTTGACAATTTTGGTTTGGGG
CTTTTGCTTCGGTCGAAGCAGATAAAACGCATGGTCTCTTCATATGTGGGAGAAAATGCA
GAATTTGAACGACAGTACTTATCTGGTGAATTAGAAGTGGAGCTGACACCACAGGGCACA
CTTGCAGAGAGGATCCGTGCAGGCGGGGCTGGAGTTCCTGCATTTTACACCCCAACAGGG
TATGGGACCCTGGTACAAGAAGGAGGATCGCCCATCAAATACAACAAAGATGGCAGTGTT
GCCATTGCCAGTAAGCCAAGAGAGGTGAGGGAGTTCAATGGTCAGCACTTTATTTTGGAG
GAAGCAATTACAGGGGATTTTGCTTTGGTGAAAGCCTGGAAGGCGGACCGAGCAGGAAAC
GTGATTTTCAGGAAAAGTGCAAGGAATTTCAACTTGCCAATGTGCAAAGCTGCAGAAACC
ACAGTGGTAGAGGTTGAAGAAATTGTGGATATTGGAGCATTTGCTCCAGAAGACATCCAT
ATTCCTCAGATTTATGTACATCGCCTTATAAAGGGAGAAAAATATGAGAAAAGAATTGAG
CGTTTATCAATCCGGAAAGAGGGAGATGGGGAAGCCAAATCTGCTAAACCTGGAGATGAC
GTAAGGGAACGAATCATCAAGAGGGCCGCTCTTGAGTTTGAGGATGGCATGTATGCTAAT
TTGGGCATAGGAATCCCTCTCCTGGCCAGCAATTTTATCAGCCCAAATATAACTGTTCAT
CTTCAAAGTGAAAATGGAGTTCTGGGTTTGGGTCCATATCCACGACAACATGAAGCTGAT
GCAGATCTCATCAATGCAGGCAAGGAAACAGTTACTATTCTTCCAGGAGCCTCTTTTTTC
TCCAGCGATGAATCATTTGCAATGATTAGAGGTGGACACGTCGATCTGACAATGCTAGGA
GCGATGCAGGTTTCCAAATATGGTGACCTGGCTAACTGGATGATACCTGGGAAGATGGTG
AAAGGAATGGGAGGTGCTATGGATTTAGTGTCCAGTGCGAAAACCAAAGTGGTGGTCACC
ATGGAGCATTCTGCAAAGGGAAATGCACATAAAATCATGGAGAAATGTACATTACCATTG
ACTGGAAAGCAATGTGTCAACCGCATTATTACTGAAAAGGCTGTGTTTGATGTGGACAAG
AAGAAAGGGTTGACTCTGATTGAGCTCTGGGAAGGCCTGACAGTGGATGACGTACAAAAG
AGTACTGGGTGTGATTTTGCAGTTTCACCAAAACTCATGCCAATGCAGCAGATCGCAAAT
TGA

• CoA_trans (PF01144 )

>Succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial
MAALKLLSSGLRLCASARGSGATWYKGCVCSFSTSAHRHTKFYTDPVEAVKDIPDGATVL
VGGFGLCGIPENLIDALLKTGVKGLTAVSNNAGVDNFGLGLLLRSKQIKRMVSSYVGENA
EFERQYLSGELEVELTPQGTLAERIRAGGAGVPAFYTPTGYGTLVQEGGSPIKYNKDGSV
AIASKPREVREFNGQHFILEEAITGDFALVKAWKADRAGNVIFRKSARNFNLPMCKAAET
TVVEVEEIVDIGAFAPEDIHIPQIYVHRLIKGEKYEKRIERLSIRKEGDGEAKSAKPGDD
VRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNITVHLQSENGVLGLGPYPRQHEAD
ADLINAGKETVTILPGASFFSSDESFAMIRGGHVDLTMLGAMQVSKYGDLANWMIPGKMV
KGMGGAMDLVSSAKTKVVVTMEHSAKGNAHKIMEKCTLPLTGKQCVNRIITEKAVFDVDK
KKGLTLIELWEGLTVDDVQKSTGCDFAVSPKLMPMQQIAN

• 3DLX

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2. Kassovska-Bratinova S, Fukao T, Song XQ, Duncan AM, Chen HS, Robert MF, Perez-Cerda C, Ugarte M, Chartrand C, Vobecky S, Kondo N, Mitchell GA: Succinyl CoA: 3-oxoacid CoA transferase (SCOT): human cDNA cloning, human chromosomal mapping to 5p13, and mutation detection in a SCOT-deficient patient. Am J Hum Genet. 1996 Sep;59(3):519-28. [PubMed:8751852 ]
3. Fukao T, Mitchell GA, Song XQ, Nakamura H, Kassovska-Bratinova S, Orii KE, Wraith JE, Besley G, Wanders RJ, Niezen-Koning KE, Berry GT, Palmieri M, Kondo N: Succinyl-CoA:3-ketoacid CoA transferase (SCOT): cloning of the human SCOT gene, tertiary structural modeling of the human SCOT monomer, and characterization of three pathogenic mutations. Genomics. 2000 Sep 1;68(2):144-51. [PubMed:10964512 ]
4. Song XQ, Fukao T, Watanabe H, Shintaku H, Hirayama K, Kassovska-Bratinova S, Kondo N, Mitchell GA: Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency: two pathogenic mutations, V133E and C456F, in Japanese siblings. Hum Mutat. 1998;12(2):83-8. [PubMed:9671268 ]