ACADM and DBT

		ACADM	DBT
Description		acyl-CoA dehydrogenase medium chain	dihydrolipoamide branched chain transacylase E2
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GO Annotations	Cellular Component	Nucleus Cytoplasm Mitochondrion Mitochondrial Matrix Axon Mitochondrial Membrane	Mitochondrion Mitochondrial Matrix Cytosol Microtubule Cytoskeleton Mitochondrial Nucleoid Branched-chain Alpha-ketoacid Dehydrogenase Complex
	Molecular Function	Acyl-CoA Dehydrogenase Activity Oxidoreductase Activity Oxidoreductase Activity, Acting On The CH-CH Group Of Donors Identical Protein Binding Flavin Adenine Dinucleotide Binding Medium-chain Fatty Acyl-CoA Dehydrogenase Activity	Protein Binding Transferase Activity Acyltransferase Activity Acyltransferase Activity, Transferring Groups Other Than Amino-acyl Groups Ubiquitin Protein Ligase Binding Dihydrolipoamide Branched Chain Acyltransferase Activity
	Biological Process	Liver Development Glycogen Biosynthetic Process Organic Acid Metabolic Process Regulation Of Gluconeogenesis Lipid Metabolic Process Fatty Acid Metabolic Process Fatty Acid Beta-oxidation Heart Development Response To Cold Carnitine Metabolic Process Post-embryonic Development Carnitine Metabolic Process, CoA-linked Fatty Acid Beta-oxidation Using Acyl-CoA Dehydrogenase Response To Starvation Carnitine Biosynthetic Process Medium-chain Fatty Acid Metabolic Process Medium-chain Fatty Acid Catabolic Process Cardiac Muscle Cell Differentiation	Branched-chain Amino Acid Catabolic Process Branched-chain Alpha-keto Acid Decarboxylation To Branched-chain Acyl-CoA
Pathways		PPARA activates gene expression mitochondrial fatty acid beta-oxidation of unsaturated fatty acids Beta oxidation of decanoyl-CoA to octanoyl-CoA-CoA Beta oxidation of octanoyl-CoA to hexanoyl-CoA	Branched-chain amino acid catabolism RHOH GTPase cycle Mitochondrial protein degradation Protein lipoylation BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV Loss-of-function mutations in DBT cause MSUD2 Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD Loss-of-function mutations in DLD cause MSUD3/DLDD Branched-chain ketoacid dehydrogenase kinase deficiency H139Hfs13* PPM1K causes a mild variant of MSUD
Drugs		Octanoyl-Coenzyme A Flavin adenine dinucleotide 3-thiaoctanoyl-CoA
Diseases		Disorders of fatty-acid oxidation, including: Medium-chain (MC) acyl-CoA dehydrogenase (AD) deficiency (MCADD); Short-chain AD deficiency (SCADD) ; Short-branched-chain AD deficiency (SBCADD) ; Very long-chain AD deficiency (VLCADD) ; Long-chain 3-hydroxyacyl CoA dehydrogenase deficiency (LCHADD) ; Trifunctional protein deficiency (TFP); Carnitine palmitoyltransferase deficiency (CPT); Carnitine-acylcarnitine translocase deficiency (CACT); Systemic primary carnitine deficiency (CDSP)	Maple syrup urine disease (MSUD)
GWAS		Daytime sleep phenotypes ( 27126917) Glycine levels ( 30837465 31070104) Metabolic traits ( 21886157) Metabolite levels ( 20037589) Serum metabolite concentrations in chronic kidney disease ( 29545352) Serum metabolite levels ( 23093944 33031748) Serum metabolite ratios in chronic kidney disease ( 29545352) Urinary metabolite levels in chronic kidney disease ( 31959995) Urinary metabolite modules (eigenmetabolites) in chronic kidney disease ( 31959995)	Lung cancer in ever smokers ( 28604730)
Interacting Genes		5 interacting genes: CALM1 CDC42 DYNC1I1 PANK1 SUMO4	1 interacting genes: OGT
Entrez ID		34	1629
HPRD ID		08447	02010
Ensembl ID		ENSG00000117054	ENSG00000137992
Uniprot IDs		A0A0S2Z366 B4DJE7 B7Z9I1 P11310 Q5T4U5	A0A7P0T9W1 P11182
PDB IDs		1EGC 1EGD 1EGE 1T9G 2A1T 4P13 8SGP	1K8M 1K8O 1ZWV 2COO 3RNM
Enriched GO Terms of Interacting Partners?		Intracellular Signaling Cassette Small GTPase-mediated Signal Transduction Angiotensin Type I Receptor Activity Bradykinin Receptor Binding Substantia Nigra Development Negative Regulation Of High Voltage-gated Calcium Channel Activity Neural Nucleus Development Regulation Of Cytokinesis Negative Regulation Of Ryanodine-sensitive Calcium-release Channel Activity Cellular Response To Type II Interferon Cytoplasmic Ribonucleoprotein Granule GBD Domain Binding Storage Vacuole Organelle Transport Along Microtubule Dihydrolipoamide Branched Chain Acyltransferase Activity Recombination Nodule Phytanic Acid Metabolic Process Pantothenate Kinase Activity Angiotensin Type II Receptor Activity Phospholipase C-activating Angiotensin-activated Signaling Pathway Renin-angiotensin Regulation Of Aldosterone Production Organelle Localization Response To Type II Interferon Nucleotide Binding Apolipoprotein A-I Receptor Binding Endothelin Receptor Signaling Pathway Involved In Heart Process Neuropilin Signaling Pathway Branched-chain Alpha-keto Acid Decarboxylation To Branched-chain Acyl-CoA Branched-chain Alpha-ketoacid Dehydrogenase Complex Methyl-branched Fatty Acid Metabolic Process Long-chain Fatty Acid Import Into Peroxisome Acetyl-CoA Binding SUMO Ligase Complex	Regulation Of Gluconeogenesis Regulation Of Glucose Metabolic Process Mitochondrial Membrane Protein N-acetylglucosaminyltransferase Complex Negative Regulation Of Non-canonical Inflammasome Complex Assembly Regulation Of Small Molecule Metabolic Process Carnitine Metabolic Process, CoA-linked Medium-chain Fatty Acyl-CoA Dehydrogenase Activity Medium-chain Fatty Acid Catabolic Process Carnitine Biosynthetic Process Protein O-acetylglucosaminyltransferase Activity Positive Regulation Of Transcription From RNA Polymerase II Promoter By Glucose Non-canonical Inflammasome Complex Assembly Positive Regulation Of Transcription By Glucose Regulation Of Transcription From RNA Polymerase II Promoter By Glucose Response To Nutrient Levels Carbon Catabolite Regulation Of Transcription Medium-chain Fatty Acid Metabolic Process Regulation Of Transcription By Glucose Carnitine Metabolic Process Fatty Acid Beta-oxidation Using Acyl-CoA Dehydrogenase Acyl-CoA Dehydrogenase Activity NSL Complex Amino-acid Betaine Metabolic Process Glycogen Biosynthetic Process Regulation Of Neurotransmitter Receptor Localization To Postsynaptic Specialization Membrane Acetylglucosaminyltransferase Activity Polysaccharide Biosynthetic Process Oxidoreductase Activity, Acting On The CH-CH Group Of Donors Sin3-type Complex Histone Acetyltransferase Complex Negative Regulation Of Stem Cell Population Maintenance Regulation Of Protein Localization To Synapse Regulation Of Receptor Localization To Synapse Regulation Of Necroptotic Process Regulation Of Rac Protein Signal Transduction Cardiac Muscle Cell Differentiation Phosphatidylinositol-3,4,5-trisphosphate Binding Cellular Response To Nutrient Regulation Of Programmed Necrotic Cell Death Response To Cold Pyroptotic Inflammatory Response Cardiocyte Differentiation Negative Regulation Of Translational Initiation Negative Regulation Of Proteasomal Ubiquitin-dependent Protein Catabolic Process Positive Regulation Of Translational Initiation Positive Regulation Of Stem Cell Population Maintenance Mitochondrion Glycogen Metabolic Process Energy Reserve Metabolic Process
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