Mitochondrial trifunctional protein

Mitochondrial trifunctional protein: Schematic demonstrating mitochondrial fatty acid beta-oxidation and effects of long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, LCHAD deficiency

Mitochondrial trifunctional protein is a protein which catalyzes several reactions in beta oxidation. It has two subunits:

HADHA

HADHB

The three functions are long-chain 3-hydroxy acyl-coenzyme A dehydrogenase, 2-enoyl coenzyme A (CoA) hydratase, and long-chain 3-ketoacyl CoA thiolase.^[1]

Pathology

Disorders are associated with:

Mitochondrial trifunctional protein deficiency

LCHAD deficiency

References

^ "Long-Chain Acyl CoA Dehydrogenase Deficiency: eMedicine Pediatrics: Genetics and Metabolic Disease". http://emedicine.medscape.com/article/945857-overview. Retrieved 2009-07-11.

External links

MeSH mitochondrial+trifunctional+protein+TP

v · Enzymes: multienzyme complexes

Photosynthesis
Photosynthetic reaction center complex proteins · Photosystem

Dehydrogenase
Pyruvate dehydrogenase complex (E1, E2, E3) · Oxoglutarate dehydrogenase (OGDH, DLST, DLD) · Branched-chain alpha-keto acid dehydrogenase complex (BCKDHA, BCKDHB, DBT, DLD)

Other
CAD (Carbamoyl phosphate synthase II, Aspartate carbamoyltransferase, Dihydroorotase) · Cholesterol side-chain cleavage enzyme · Cytochrome b6f complex · Electron transport chain · Fatty acid synthetase complex · Glycine decarboxylase complex · Mitochondrial trifunctional protein (HADHA, HADHB) · Phosphoenolpyruvate sugar phosphotransferase system · Polyketide synthase · Sucrase-isomaltase complex · Tryptophan synthase

v · Metabolism: lipid metabolism / fatty acid metabolism · triglyceride and fatty acid enzymes

Synthesis

Malonyl-CoA synthesis

ATP citrate lyase · Acetyl-CoA carboxylase

Fatty acid synthesis/
Fatty acid synthase

Beta-ketoacyl-ACP synthase · Β-Ketoacyl ACP reductase · 3-Hydroxyacyl ACP dehydrase · Enoyl ACP reductase

Fatty acid desaturases

Stearoyl-CoA_desaturase-1

Triacyl glycerol

Glycerol-3-phosphate dehydrogenase · Thiokinase

Degradation

Acyl transport

Carnitine palmitoyltransferase I · Carnitine-acylcarnitine translocase · Carnitine palmitoyltransferase II

Beta oxidation

General

Acyl CoA dehydrogenase (ACADL, ACADM, ACADS, ACADVL, ACADSB) · Enoyl-CoA hydratase

MTP: HADH · HADHA · HADHB
Acetyl-CoA C-acyltransferase

Unsaturated

Enoyl CoA isomerase · 2,4 Dienoyl-CoA reductase

Odd chain

Propionyl-CoA carboxylase

Other

Hydroxyacyl-Coenzyme A dehydrogenase

To acetyl-CoA

Malonyl-CoA decarboxylase

Aldehydes

Long-chain-aldehyde dehydrogenase

M: MET

mt, k, c/g/r/p/y/i, f/h/s/l/o/e, a/u, n, m

k, cgrp/y/i, f/h/s/l/o/e, au, n, m, epon

m(A16/C10),i(k, c/g/r/p/y/i, f/h/s/o/e, a/u, n, m)

v · d · eMitochondrial proteins

Outer membrane

fatty acid degradation (Carnitine palmitoyltransferase I, Long fatty acyl CoA synthetase)

tryptophan metabolism (Kynureninase)
monoamine neurotransmitter metabolism (Monoamine oxidase)

Intermembrane space
Adenylate kinase - Creatine kinase

Inner membrane

oxidative phosphorylation (Coenzyme Q - cytochrome c reductase, Cytochrome c, NADH dehydrogenase, Succinate dehydrogenase)

pyrimidine metabolism (Dihydroorotate dehydrogenase)

mitochondrial shuttle (Malate-aspartate shuttle, Glycerol phosphate shuttle)
other (Glutamate aspartate transporter, Glycerol-3-phosphate dehydrogenase, ATP synthase, Carnitine palmitoyltransferase II, Uncoupling protein)

Matrix

citric acid cycle (Citrate synthase, Aconitase, Isocitrate dehydrogenase, Oxoglutarate dehydrogenase, Succinyl coenzyme A synthetase, Fumarase, Malate dehydrogenase)

anaplerotic reactions (Aspartate transaminase, Glutamate dehydrogenase, Pyruvate dehydrogenase complex)

urea cycle (Carbamoyl phosphate synthetase I, Ornithine transcarbamylase, N-Acetylglutamate synthase)

alcohol metabolism (ALDH2)
PMPCB

Other/to be sorted

steroidogenesis (Cholesterol side-chain cleavage enzyme, Steroid 11-beta-hydroxylase, Aldosterone synthase), Frataxin
Mitochondrial membrane transport protein (Mitochondrial permeability transition pore, Mitochondrial carrier)

Mitochondrial DNA

Complex I (MT-ND1, MT-ND2, MT-ND3, MT-ND4, MT-ND4L, MT-ND5, MT-ND6) - Complex III (MT-CYB) - Complex IV (MT-CO1, MT-CO2, MT-CO3)

ATP synthase (MT-ATP6, MT-ATP8)
tRNA (MT-TA, MT-TC, MT-TD, MT-TE, MT-TF, MT-TG, MT-TH, MT-TI, MT-TK, MT-TL1, MT-TL2, MT-TM, MT-TN, MT-TP, MT-TQ, MT-TR, MT-TS1, MT-TS2, MT-TT, MT-TV, MT-TW, MT-TY)

see also mitochondrial diseases
B strc: edmb (perx), skel (ctrs), epit, cili, mito, nucl (chro)

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