Proteínas de la matriz mitocondrial

Las proteínas de la matriz mitocondrial se exponen en la siguiente tabla, a excepción de un grupo relacionado con el dogma central en la mitocondria ( replicación, transcripción y traducción ) que lo hemos dispuesto en la tabla de proteínas de la matriz relacionadas con  replicación, transcripción y traducción


Ha sido encontrada en humano y es codificada por un gen nuclear
Ha sido encontrada en humano y es codificada por un gen mitocondrial
No ha sido encontrada en humano y es codificada por un gen nuclear


Nombre de la Proteína  Símbolo Nombre del Gen Posición Citogenetica
L-3-hidroxiacil-CoA:NAD oxidoreductasa  rata
L-Malato:NAD+ oxidoreductasa (iso)   "diversas especies, marker enzima, some bound to M face of im"
LS-Isocitratao:NAD oxidoreductasa (decarboxilating)  diversas especies
LS-Isocitratao:NADP oxidoreductasa (decarboxilating)  diversas especies
1-pirrolina-5-carboxilato:NAD(+) oxidoreductasa  rata
L-Glutamato NAD(P) oxidoreductasa (deaminating)  diversas especies
Carbomyl fosfato:L-ornithina carbomyl transferasa  "rata, rabbit liver"
Acetil-CoA:carnitina O-acetiltransferasa  "pig heart, rata heart, pigeon muscle, sheep tissues"
L-Aspartato:2-oxoglutaratao aminotransferasa (iso)  diversas especies
ATP:nucleosido difosfato fosfotransferasa  rata
Malonil-CoA decarboxilasa   
Citratao oxaloacetato-lyasa (CoA-acetilating)  diversas especies
Acetato:CoA ligasa (AMP)  "rata, ruminants"
Acid:CoA ligasa (AMP)  "ox, rata"
Succinato:CoA ligasa (GDP)  "pig heart, rata"
piruvato:CO2 ligasa (ADP)  diversas especies
Propionil-CoA:CO2 ligasa (ADP)  "guinaa pig heart, ox, rata"
Carbamoil fosfato synthetasa I  liver
Aspartato aminotransferasa   
glutamato dehidrogenasa  "liver and kidney especially NAD or NADP, (plants, & microbes, often NAD- or NADP-specific)"
calcio-dependiente fosfolipasa   
alanina aminotransferasa   
ornithina aminotransferasa   
5-aminolevulinato synthasa   
Superoxido dismutasa (Mn form) (SOD)  "isoenzima of cytoplasm form is from different ancestral gene, only known example, but homologous to prokaryotic SOD"
Isocitratao dehidrogenasa  some bound to M face of im; Mn2+
ATP-dependiente proteasaLON-PEN"Lon, ATP-dependiente proteasa (homolog of bacterial Lon)" no asignado 

human hippocampus cDNA library

ATP-dependiente proteasaP26S4-PEN"proteasa, 26S, ubiquitin/ATP-dependiente, subunit 4, 51kDa" no asignado
Nucleosidodifosfato kinasa   
GTP-AMP fosfotransferasa   
Glycerol-3-fosfato dehidrogenasa   
GTP-specific succinil-CoA synthetasa  "extrahepatic cells(?), functions during ketone body formation (!Section 5.2.5) and in heme biosynthesis (!Section 9.5.1)"
Malic enzima [aka Malato dehidrogenasa (oxaloacetato-decarboxilating)]  "malato to piruvato, mito in intestinal, brain, and tumor cells where leads to enhanced unregulatod cholesterol synthesis, but in most vert. tissues is cytosolic"
Glutaminasa  "aka L-Glutamina amidohidrolasa (iso). glutamina to glutamato and ammonia, intestina, tumor cells, rata."
Catalasa  "mainly in peroxisomes, but also at least in rata heart"
Glutathione peroxidasa  "rata mainly in cytosol, but ca 15% activity in matrix; attack peroxidized fa's releasad from membrane by fosfolipasa A2"
2-oxoglutaratao dehidrogenasa (decarboxilating)  E1
Dihidrolipoamida succiniltransferasa  E2
Dihidrolipoamida dehidrogenasa  "E3, possibly identical to piruvato DH subunit; only deficiency in TCA cicle enzimas, infantile encefalmiopati"
Aconitasa  "isoenzima in cytosol, aka aconitato hydratasa, Citratao (isocitratao) hidro-lyasa"
"Isocitratao dehidrogenasa, NAD(P)-linked "  NADP-linked also here and in cytosol
Mitocondrial L-Malato dehidrogenasa  "heart muscle, 66-70 kDal, with 2 subunits, reversible and equilibrium strongly in favor of malato, but rapid removal of oxaloacetato by condensation with acetilCoA to form citratao shifts equilibrium"
Citrato synthasa { (re) (si) }  "formerly citratao condensing enzima, heart form has 2 identical 100kDal subunits"
GABA-2-OG aminotransferasa  "formation and releasa of GABA by presynaptic cells, uptake and metabolism of GABA by glial cells"
Succinato semialdehyde dehidrogenasa (NAD+)  "In Brain, up to 1/3 of 2-oxoglutaratao formed by IDH is removed to glutamato, then to cytosol, udergoing decarboxilation to GABA, some GABA is taken into mito and metabolized to succinato"
Succinato semialdehyde dehidrogenasa (NADP+)   
"Succinil-CoA Synthetasa, GDP-forming"  "alfa subunit, 33.5 kDal, site of fosforilation"
"Succinil-CoA Synthetasa, ADP-forming"  "aka Succinato:CoA ligasa (ADP). Heart muscle, 80 kDal, catalytic center at interface of two subunits. artichoke tuber."
Succinil-CoA Synthetasa  "aka succinato thiokinasa, "
mitocondrial Aspartato aminotransferasa   
Anchor proteins  "isoenzima in cytoplasm from same gene, mt isoenzima not acetilatod at N terminus"
Succinato dehidrogenasa  "ferriflavoprotein, covalently bound FAD prosthetic group, tightly bound to inner membrane, catalytic center facing matrix space, part of complex II (succinato-Q reductasa)"
Fumarasa  "aka fumaratao hydratasa, akaL-Malato hidro-lyasa. heart muscle form with 4 identical subunits combina 194 kDal, a few casas of deficiency"
Transhidrogenasa  "transfers reducing equivalents between NADf to NAD+, metabolizing TCA, involved at isocitrataoDH"
Acetil-CoA synthetasa   
Propionil-CoA synthetasa  liver
Propionil-CoA carboxilasa  "liver, biotin containing"
Metilmalonil-CoA racemasa  liver
Metilmalonil-CoA mutasa  "aka 2-Metilmalonil-CoA CoA-carbonilmutasa, liver, one of the few requiring 5'-deoxiadenosylcobalamin, from vitamin B12"
piruvato carboxilasa  "exclusively matrix space, acts with PEPCK to convert P to PEP in liver and kidney "
Butiril-CoA synthetasa   
Short-chain fatty acil-CoA Dehidrogenasa   
Medium-chain fatty acil-CoA dehidrogenasa   
Long-chain fatty acil-CoA dehidrogenasa   
Enoil-CoA hydratasa   
beta-hidroxiacil-CoA dehidrogenasa  aka 3-hidroyacil-CoA dehidrogenasa
AcetilCoA aciltransferasa (thiolysis)  "aka acil-CoA:acetil-CoA aciltransferasa aka ACEtil-COA C-acilTRANSFERasa aka BETA-KETOTHIOLasa aka 3- KETOacil-COA THIOLasa. mito and peroxisome have specific forms. Diseasa: PSEUDO-ZELLWEGER SYNDROME. Locusta flight muscle, rata heart, pigeon muscle"
Branched-chain 2-oxo acid dehidrogenasa complex  "aka 2-Oxo-acid carboxi-lyasa, rata kidney, irreversible, similar to PDH complex"
fosfoenolpiruvato carboxikinasa (matrix PEPCK)  "cytosolic and matrix space (species & tissue differences in !5:38) to undergo net oxidation, beta-ox intermediatos converted to acetilCoA thru this enzima"
short-chain fatty acil-CoA synthetasa  rata
"2,4-dienoil-CoA reductasa (NADf)"  NADP-linked
"cis-3,trans-2-enoil-CoA isomerasa"DCIdodecanoil-coenzima A delta-isomerasa 16p13.3
Acetil-CoA acetiltransferasa  "liver, extrahepatic cells"
hidroximetilglutaril-CoA lyasa  aka 3-hidroxi-3-metilglutaril-CoA acetoacetato lyasa. rata
hidroximetilglutaril-CoA synthasa  1p12-p13


aka hidroximetilglutaril-CoA reductasa, aka 3-hidroxi-3-metilglutaril-CoA acetoacetil-CoA-lyasa (CoA acetilating). ratao determining enzima of cholesterol synthesis, in tumors increasad cholesterol content, enzima loses cholesterol feedback. rata."

Fatty acil-CoA hidrolasa  liver
NAD-linked D-3-hidroxibutiratao dehidrogenasa  nonruminant liver (cytosolic in ruminants)
D-3-hidroxibutiratao dehidrogenasa  extrahepatic cells
3-oxoacid CoA-transferasa  "pig heart, extrahepatic cells, aka succinil-CoA-actoacetato transferasa, aka Succinil-CoA:3-oxo-acid CoA-transferasa"
Aminotransferasas  rata
Glutamato dehidrogenasa  "rata, brain ammonia toxicity probably due to its effect on this enzima's reaction equilibrium"
"Glutamato dehidrogenasa, NAD(P)+"   
"Glutamato dehidrogenasa, NADP+"   
Carbamoilfosfato synthetasa I (urea cicle enzima)  rata
Ornithina transcarbamylasa (urea cicle enzima)  rata
Alanina aminotransferasa  "aka L-Alanina:2-oxoglutaratao aminotransferasa, aka ALANina AMINOTRANSFERasa aka GLUTAMIC--pirUVIC TRANSAMINasa aka GLUTAMIC--ALANina TRANSAMINasa. rata, pig heart, intestinas, oxidation of gluamato to alanina"
Carbamoil fosfato synthetasa I (ammonia)  "apparently ratao limiting step in urea synthesis, activity dependiente on ammonia, ornithina, and N-acetilglutamato, allosteric activator, in elasmobranchs is gln-dependiente (CPSIII), in amfibs & mammals depends on ammonia (CPSI), in ureotelic animals"
Glutamina synthetasa  "analogous to CPE in uricotelic animals eg., birds and some reptiles, glutaminarataher than citrullina is transported out and converted to uric acid"
Arginasa  "releasas urea; in elasmobranchs is mitocondrial, in amfibs and mammals is cytosolic"
Ornithina transcarbamylasa   
Carbonic anhydrasa  "320kDal, two similar subunits, Requires MgATP and free Mg+2,15-25% of total matrix-space protein in liver of ureotelic animals, apparently absent from heart, brain and kidney but present in liver and skeletal muscle"
a glutamato-glutamato gamma-semialdehyde dehidrogenasa  to replenish ornithina along with next enzima in liver
a glutamato gamma-semialdehyde aminotransferasa  replenishes ornithina
N-acetilglutamato synthasa  "uses acetil-CoA and glutamato, allosterically activatod by arginina"
piruvato dehidrogenasa (decarboxilating)=E1   "bovina heart, (2 subunits, 41 kD, 36 kD"
Dihidrolipoamida acetiltransferasa=E2   "bovina heart, 52kD single chain"
Dihidrolipoamida dehidrogenasa=E3   "bovina heart, 110 kD dimer of 2 55kD monomers"
piruvato dehidrogenasa kinasa  "bovina heart, (2 subunits, 48, 45 kD)"
piruvato dehidrogenasa fosfato fosfatasa  "bovina heart, (2 subunits, 97, 50 kD)"
Carnitina-palmitoil-CoA transferasa  Is it mito or cyto???
"Carnitina palmitoiltransferasa (CPT II) (internal, latont)"CPT1carnitina palmitoiltransferasa 1 1p32
glycerol 3-fosfato dehidrogenasa  "flavoprotein, mammalian tissue and insect flight muscle"
Cholina dehidrogenasa  "cholina to betaina aldehyde, linked directly to respirataory chain"
(acceptor) Aldehyde dehidrogenasa I (uM Km)  "most mammalian livers, low Km(uM), principle enzima oxidizing acetaldehyde during ethanol oxidation, betaina aldehyde to betaina, a 1 carbon metil or hidroximetil donor, linked directly to respirataory chain"
Aldehyde dehidrogenasa II (mM Km)  "most mammalian livers & human brain, high Km(mM), glutamic-gamma-semialdehyde dehidrogenasa in human liver, succinato-semialdehydehyde dehidrogenasa in brain "
"Aldehyde dehidrogenasa, NAD+"   
"Aldehyde dehidrogenasa, NADP+"   
"Aldehyde dehidrogenasa, NAD(P)+"   
Sarcosina dehidrogenasa  "sarcosina to glycina, covalently bound FAD and tightly cound folato"
Dimetilglycina dehidrogenasa  "rata, dimetilglycina to sarcosina, covalently bound FAD and tightly bound folato"
glycina synthasa  "aka Glycina-cleavage enzima. liver form has 4 protein components in multienzima complex similar to 2-oxo acid dehidrogenasa, forms CO2, NH4+, metilene tetrahidrofolato, NADH, H+ form glycina"
Serina hidroximetiltransferasa  "aka serina hidroximetilasa, isozyme in cytosol, glycina+metilene tetrahidrofolato to serina and tetrahidrofolato"
glycina aminotransferasa   
2-metil branched chain enoil-CoA reductasa  "anaerobic, in Ascaris?, heme synthesis"
5-aminolevulinato synthasa  "functions in formation of porfyrin nucleus, heme synthesis"
N-acetilglutamato synthetasa  regulatod by arginina transport into mitos
Mitocondrial Processing Peptidasa (MPP)  "ca 50 kDal, 26% aa identity with PEP, protein family includes subunits I and II of complex III"
Processing-enhancing protein (PEP)  "ca 50 kDal, 26% aa identity with MPP, protein family includes subunits I and II of complex III"
Hsp60  matrix space analogue of cytosolic Hsp70
Dihidroorotato-ubiquinone reductasa   "on C-face of inner membrane, involved in pirimidina syntehsis "
NAD-linked Dehidrogenasa  succinato to succinato semialdehyde
NADP-linked reductasa  "succinato semialdehyde to 4-hidroxibutiratao, in 4-Hb acidemia"
rhodanese aka Thiosulfato sulfurtransferasa  "aka thiosulfato-cyanide sulfurtransferasa. widely distributed in mammalian tissues, forms sulfite and thiocyanato, Fe-S center synthesis. serum albumin-sulfane complex prob. prim. cyanide disposal system. most vital site of cyanide action is COX of CNS"
5-aminolevulinato synthasa (ALA synthasa)  first and ratao-determining step from succinilCoA and glycina. Final three steps by enzimas associatod with the inner membrane
6N-trimetillysina-2-oxoglutaratao dioxigenasa   
Glutamato dehidrogenasa  liver
Succinato dehidrogenasa  rata
citratao synthasa  aka CITratao (SI)-SYNTHasa aka CONDENSING enzima aka CITratao CONDENSING enzima aka CITROGENasa aka OXALOACETato
L-lactato:NAD oxidoreductasa(iso)  several
6-fosfo-D-gluconato:NAD oxidoreductasa (iso)  rata “latont”
D-Glucose-6-fosfato:NADP oxidoreductasa (iso)  aka D-Glucose-6-fosfato 1-dehidrogenasa aka G6PD. rata
3(or 17)-beta -hidroxisteroid:NAD(P) oxidoreductasa (NAD)   "3(or 17)-beta -hidroxisteroid dehidrogenasa, guinaa pig liver, ox adrenal"
21-hidroxisteroid-NAD oxidoreductasa  sheep liver
24-Dehidrocholestreol reductasa  rata
’-pirrolina-5-carboxilato:NAD oxidoreductasa  Locusta muscle
L-Lactato:cytocrome c oxidoreductasa  yeast
Cholina:(acceptor) oxidoreductasa  aka cholina dehidrogenasa. rata
Betaina aldehyde:NAD oxidoreductasa  aka Betaina-aldehyde dehidrogenasa. rata
Aldehyde:O2 oxidoreductasa  pig liver
piruvato:lipoato oxidoreductasa (acceptor acetilating)  pig heart
Butiril-CoA:cytocrome c oxidoreductasa  "ox, pig liver"
mitocondrial protein P1   
acil-CoA:cytocrome c oxidoreductasa  "rata, pig liver"
Coproporfyrinogen oxidasa (decarboxilating) not  "ox, guinaa, pig liver"
Succinato cytocrome c reductasa not  rata
Diamina:O2 oxidoreductasa (deaminating)  "rabbit liver, pig kidney"
Dihidrofolato reductasa  "aka 5,6,7,8-Tetrahidrofolato:NAD(P) oxidoreductasa aka tetrahidrofolato dehidrogenasa. rata"
"7,8-Dihidrofolato:NADP oxidoreductasa"  rata
Sarcosina:O2 oxidoreductasa (demetilating)  "aka sarcosina oxidasa. rata, ""soluble"""
NADH2:cytocrome c oxidoreductasa  diversas especies
NADf2:cytocrome c oxidoreductasa  "diversas especies, Ascaris muscle"
NADH2:lipoamida oxidoreductasa  rata
NADH2:(acceptor) oxidoreductasa  "rata, BRAIN"
Ammonia:(acceptor) oxidoreductasa  aka hidroxiLAMina REDUCTasa; liver
Sulfite:O2 oxidoreductasa  "aka sulfite oxidasa. rata, ox liver"
Donor:H2O2 oxidoreductasa  peroxidasa aka myeloperoxidasa. ascites cells
Iodina:H2O2 oxidoreductasa  "iodide peroxidasa aka iodotirosina deiodasa aka iodinasa aka tiroid peroxidasa, sheep tiroid"
L-Serina:tetrahidrofolato 10-hidroximetil transferasa (iso)  "rata, rabbit liver"
Carbomyl fosfato:L-aspartato carbomyl transferasa  "aka ASPARTato CARBAMoilTRANSFERasa aka ASPARTato TRANSCARBAMYLasa aka CARBAMYLASPARTOTRANSKINasa aka ATCasa. rata, rabbit liver"
Acetil-CoA:acetil-CoA acetiltransferasa  aka ACEtil-COA C-ACEtilTRANSFERasa aka ACETOACEtil-COA THIOLasa. Diseasa: ALfA-MEtiLACETOACETICACIDURIA. Locusta flight muscle
Acetk-CoA:dihidrolipoato S-acetiltransferasa  aka DIhidroLIPOamida S-ACEtilTRANSFERasa aka LIPOato ACEtilTRANSFERasa aka THIOLTRANSACEtilasa A.
acil-CoA:glycina N-aciltransferasa  "aka GLYCina N-acilTRANSFERasa. ox, rata"
Succinil-CoA:glycina succiniltransferasa  "rata and kidney, NOT heart NOR brain"
L-tirosina:2-oxoglutaratao aminotransferasa  aka tirOSina AMINOTRANSFERasa aka tirOSina TRANSAMINasa. Diseasa: tirOSinaMIA II (RICHNER-HANHART SYNDROME). rata
4-Aminobutiratao aminotransferasa (iso)  aka 4-AMINOBUtiratao AMINOTRANSFERasa aka GAMMA-AMINO-N- BUtiratao TRANSAMINasa aka GABA TRANSAMINasa aka BETA- ALANina--OXOGLUTAratao AMINOTRANSFERasa Diseasa: GABA- TRANSAMINasa DEFICIENCY. rata BRAIN
L-Alanina:glyoxilato aminotransferasa (iso)  "mammalian liver, kidney"
ATP:D-hexose 6-fosfotransferasa (types I & II)  aka HEXOKINasa aka GLUCOKINasa aka HEXOKINasa TYPE IV. Diseasa: HEMOLYTIC ANEMIA DUE TO HEXOKINasa DEFICIENCY. diversas especies
ATP:riboflavin 5'-fosfotransferasa  aka RIBOFLAVIN KINasa aka FLAVOKINasa. rata
ATP:piruvato fosfotransferasa  aka pirUVato KINasa aka fOSfOENOLpirUVato KINasa aka fOSfOENOL TRANSfOSfOrilasa. Diseasa:HEMOLYTIC ANEMIA DUE TO pirUVato KINasa DEFICIENCY. rabbit muscle
ATP:carbamato fosfotransferasa  aka CARBAMato KINasa. rata
Carbamoil fosfato synthasa  Inner membrane


ATP:nucleosido difosfato fosfotransferasa  "artichoke, ox heart, liver"
Nucleosidotrifosfato:RNA nucleotidyltransferasa  "rata, S. cerevisiae"
Deoxinucleosidotrifosfato:DNA deoxinucleotidyltransferasa  "yeast, rata"
"CDP cholina:1,2-diglyceride cholina fosfotransferasa"  "Tetrahymena, rata BRAIN, chicken liver"
Adenil ciclasa  "rata, BRAIN"
Palmitoil-CoA hidrolasa  calf liver
Deoxiribonucleato oligonucleotido-hidrolasa  rata
Trehalose 1-glucohidrolasa  Sarcofaga f.m. MEM.
Carboxipeptidasa A  rata
Cathepsin A  rata
L-Glutamato 1-carboxi-lyasa (iso)  "mouse, human BRAIN"
Orthofosfato:oxaloacetato carboxi-lyasa (fosforilating)  "Bryofyllum, cauliflower and pea seedlings"
GTP:oxaloacetato carboxi-lyasa (transfosforilating)(iso)  diversas especies
ATP-citratao lyasa  rata
3-hidroxiacil-CoA 3-epimerasa  rata
3-ketosteroid delta4-delta5-isomerasa  human placenta
delta3-cis-delta2-trans-enoil-CoA isomerasa  rata
Amino acid:sRNA ligasas (AMP)   
Octanoato CoA ligasa  ox liver
Benzoil-CoA synthetasa  "guinaa pig, rata"
delta-Aminolaevulinato synthetasa  "aka 5-aminolevulinato synthasa, rata"
Sarcosina dehidrogenasa  rata
Thiamina pirofosfokinasa  not sure it is mitocondrial
Octanoiltransferasa  aka octanoil-CoA:L-canitina O-octanoiltransferasa
Electron-transferring flavoprotein (ETF)   
Dicarboxilato carrier  "plant mitocondria, to transport in malato, from reduction of oxaloacetato, formed from PEP by PEP carboxilasa"
PEP carboxilasa  "in plant cytosol, forming oxaloacetato from PEP, Mg+2"
ETF dehidrogenasa ferriflavoprotein   
Q-binding apoproteins  evidence for existence largely circumstantial
QPn  "locatod in complex I, unknown MW, ?site of Q reduction and rotenone and piericidin binding"
QPs  "complex II, 15 kDal, reconstitutes with succinato to form s-Q reductasa"
QPc  "complex III, 15 kDal, reconstitutes with a deficient prep (bc1-IV complex of King) to form QH2-cyto c reductasa. Two QPc molecules per cyto c1 are thought to be present. they might function as the Qin and Qout centers of the Q-cicle in complex III"
"ETF, electron-transferring flavoprotein"  reoxidized by separatao ETF-ubiquinone oxidoreductasa
ETF-ubiquinone oxidoreductasa   
Iron-sulfur protein (FeSIII-Rieske)   
Iron-sulfur protein  FeSs-3 center (4Fe4S structure)
ETF dehidrogenasa  FeSetf center (4Fe4S structure)
a-type cytocromes  heme group contains formyl and hidrocarbon side chains not in other cytocromes
L-lactato:cytocrome c reductasa  aka cytocrome b2
D-lactato:cytocrome c reductasa  in yeast mitos
Alternative oxidasa  "cyanide-insensitive, thermolabile oxidasa activity of many plant mitos, abolished by quinone extraction and restored by adding ubiquinones"
Proton-linked Pi carrier   
Proton-linked piruvato Carrier   
Proton-linked Glutamato Carrier   

El visitante puede encontrar información adicional de cada gen y proteína en MitoDat database en, MitoDat database recopila la información sobre proteínas mitocondriales actualizada por S. Zullo, Ph. D.  y donde puede obtener otros valiosos datos incluidas las referencias bibliográficas de cada proteína.

La anterior tabla será traducida en etapas sucesivas al español, y será completada con otras aportaciones.

cadena H

pseudogenes mitocondriales

genes de tRNAs

genes de mRNAs

cadena transportadora

cadena L

nomenclatura aminoácidos

código estandar

código mitocondrial

genes de rRNAs

secuencias de DNA

expresión del mitDNA

mitobase proteica

el mitomapa


herramientas de cálculo

terapia génica y ética


la mitocondria humana

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