Showing Protein Dihydrolipoyl dehydrogenase, mitochondrial (HMDBP00054)

• 5283
• HMDBP03598

1. Dihydrolipoamide dehydrogenase
2. Glycine cleavage system L protein

• 2-aminoadipic 2-oxoadipic aciduria
• 2-ketoglutarate dehydrogenase complex deficiency
• 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
• 3-Phosphoglycerate dehydrogenase deficiency
• Beta-Ketothiolase Deficiency
• Citrate cycle (TCA cycle)
• Citric Acid Cycle
• Congenital lactic acidosis
• Dihydropyrimidine Dehydrogenase Deficiency (DHPD)
• Dimethylglycine Dehydrogenase Deficiency
• Dimethylglycine Dehydrogenase Deficiency
• Fumarase deficiency
• Glutaminolysis and Cancer
• Glutaric Aciduria Type I
• Glycine, serine and threonine metabolism
• Glycine, serine and threonine metabolism
• Glycolysis / Gluconeogenesis
• Hyperglycinemia, non-ketotic
• Hyperlysinemia I, Familial
• Hyperlysinemia II or Saccharopinuria
• Isobutyryl-coa dehydrogenase deficiency
• Isovaleric acidemia
• Isovaleric Aciduria
• Leigh Syndrome
• Lysine degradation
• Maple Syrup Urine Disease
• Methylmalonate Semialdehyde Dehydrogenase Deficiency
• Methylmalonic Aciduria
• Mitochondrial complex II deficiency
• Nitrogen metabolism
• Non Ketotic Hyperglycinemia
• Primary hyperoxaluria II, PH2
• Propionic Acidemia
• Pyridoxine dependency with seizures
• Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency)
• Pyruvate Dehydrogenase Complex Deficiency
• Pyruvate dehydrogenase deficiency (E2)
• Pyruvate dehydrogenase deficiency (E3)
• Pyruvate kinase deficiency
• Pyruvate metabolism
• Pyruvate metabolism
• Saccharopinuria/Hyperlysinemia II
• Sarcosinemia
• The oncogenic action of 2-hydroxyglutarate
• The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria
• The Oncogenic Action of Fumarate
• The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria
• The Oncogenic Action of Succinate
• Threonine and 2-Oxobutanoate Degradation
• Valine, leucine and isoleucine degradation
• Valine, leucine and isoleucine degradation
• Warburg Effect

1. Mitochondrion matrix

>1530 bp
ATGCAGAGCTGGAGTCGTGTGTACTGCTCCTTGGCCAAGAGAGGCCATTTCAATCGAATA
TCTCATGGCCTACAGGGACTTTCTGCAGTGCCTCTGAGAACTTACGCAGATCAGCCGATT
GATGCTGATGTAACAGTTATAGGTTCTGGTCCTGGAGGATATGTTGCTGCTATTAAAGCT
GCCCAGTTAGGCTTCAAGACAGTCTGCATTGAGAAAAATGAAACACTTGGTGGAACATGC
TTGAATGTTGGTTGTATTCCTTCTAAGGCTTTATTGAACAACTCTCATTATTACCATATG
GCCCATGGAAAAGATTTTGCATCTAGAGGAATTGAAATGTCCGAAGTTCGCTTGAATTTA
GACAAGATGATGGAGCAGAAGAGTACTGCAGTAAAAGCTTTAACAGGTGGAATTGCCCAC
TTATTCAAACAGAATAAGGTTGTTCATGTCAATGGATATGGAAAGATAACTGGCAAAAAT
CAAGTCACTGCTACGAAAGCTGATGGCGGCACTCAGGTTATTGATACAAAGAACATTCTT
ATAGCCACGGGTTCAGAAGTTACTCCTTTTCCTGGAATCACGATAGATGAAGATACAATA
GTGTCATCTACAGGTGCTTTATCTTTAAAAAAAGTTCCAGAAAAGATGGTTGTTATTGGT
GCAGGAGTAATAGGTGTAGAATTGGGTTCAGTTTGGCAAAGACTTGGTGCAGATGTGACA
GCAGTTGAATTTTTAGGTCATGTAGGTGGAGTTGGAATTGATATGGAGATATCTAAAAAC
TTTCAACGCATCCTTCAAAAACAGGGGTTTAAATTTAAATTGAATACAAAGGTTACTGGT
GCTACCAAGAAGTCAGATGGAAAAATTGATGTTTCTATTGAAGCTGCTTCTGGTGGTAAA
GCTGAAGTTATCACTTGTGATGTACTCTTGGTTTGCATTGGCCGACGACCCTTTACTAAG
AATTTGGGACTAGAAGAGCTGGGAATTGAACTAGATCCCAGAGGTAGAATTCCAGTCAAT
ACCAGATTTCAAACTAAAATTCCAAATATCTATGCCATTGGTGATGTAGTTGCTGGTCCA
ATGCTGGCTCACAAAGCAGAGGATGAAGGCATTATCTGTGTTGAAGGAATGGCTGGTGGT
GCTGTGCACATTGACTACAATTGTGTGCCATCAGTGATTTACACACACCCTGAAGTTGCT
TGGGTTGGCAAATCAGAAGAGCAGTTGAAAGAAGAGGGTATTGAGTACAAAGTTGGGAAA
TTCCCATTTGCTGCTAACAGCAGAGCTAAGACAAATGCTGACACAGATGGCATGGTGAAG
ATCCTTGGGCAGAAATCGACAGACAGAGTACTGGGAGCACATATTCTTGGACCAGGTGCT
GGAGAAATGGTAAATGAAGCTGCTCTTGCTTTGGAATATGGAGCATCCTGTGAAGATATA
GCTAGAGTCTGTCATGCACATCCGACCTTATCAGAAGCTTTTAGAGAAGCAAATCTTGCT
GCGTCATTTGGCAAATCAATCAACTTTTGA

• Pyr_redox (PF00070 )
• Pyr_redox_2 (PF07992 )
• Pyr_redox_dim (PF02852 )

>Dihydrolipoyl dehydrogenase, mitochondrial
MQSWSRVYCSLAKRGHFNRISHGLQGLSAVPLRTYADQPIDADVTVIGSGPGGYVAAIKA
AQLGFKTVCIEKNETLGGTCLNVGCIPSKALLNNSHYYHMAHGKDFASRGIEMSEVRLNL
DKMMEQKSTAVKALTGGIAHLFKQNKVVHVNGYGKITGKNQVTATKADGGTQVIDTKNIL
IATGSEVTPFPGITIDEDTIVSSTGALSLKKVPEKMVVIGAGVIGVELGSVWQRLGADVT
AVEFLGHVGGVGIDMEISKNFQRILQKQGFKFKLNTKVTGATKKSDGKIDVSIEAASGGK
AEVITCDVLLVCIGRRPFTKNLGLEELGIELDPRGRIPVNTRFQTKIPNIYAIGDVVAGP
MLAHKAEDEGIICVEGMAGGAVHIDYNCVPSVIYTHPEVAWVGKSEEQLKEEGIEYKVGK
FPFAANSRAKTNADTDGMVKILGQKSTDRVLGAHILGPGAGEMVNEAALALEYGASCEDI
ARVCHAHPTLSEAFREANLAASFGKSINF

• 1ZMC
• 1ZMD
• 1ZY8
• 2F5Z
• 3RNM

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