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Table 1 Overview of diseases associated with the mitochondrial proteome

From: Comparative mitochondrial proteomics: perspective in human diseases

Organ

Disease (Researcher)

Analytical method

Major proteins identified

Functional distribution

Primary Significance

Up-regulated

Down-regulated

Nervous system

      
 

Alzheimer's disease (AD) (Lovell MA.)

ICAT, 2D-LC/MS/MS

ATP synthase alpha chain

 

OXPHOS

Cells undergoing Aß -mediated apoptosis increase synthesis of proteins essential for ATP production and efflux to maintain metabolic functions.

   

Pyruvate kinase, M1 isozyme

 

glycolysis

 
   

Glyceraldehyde

3 phosphate dehydrogenase

 

energy production

 
   

Cofilin

 

control of actin polymerization/depolymerization

 
   

Na+/K + -transporting ATPase a-3 chain

 

ATP production

 
   

VDAC 1 and 3

 

apoptosis

 
   

Dihydropyrimidinase- related protein-1 (DRP-1)

 

axon guidance, invasive growth and cell migration

 
 

Multiple sclerosis (MS) (Broadwater L.)

SELDI-TOF-MS

 

Cytochrome c oxidase subunit 5b (COX5b)

component of Complex IV of the electron transport chain

Proteins identified would be used as neuroprotective therapeutic targets for MS.

   

Hemoglobin β-chain

 

oxygen transport

 
   

Myelin basic protein (MBP)

 

component of the myelin membrane in the CNS

 
   

Creatine kinase (CKB)

 

creatine metabolic process

 
 

Neural degeneration (Pienaar IS.)

2DE, ESI-QUADTOF/MS

 

Protein disulfide isomerase (PDI)

folding

Alteration of mitochondrial function may contribute to the beneficial effects associated with statin use.

   

Heat shock proteins

 

protein assembly and folding

 
    

Dehydrogenase antiporter

transportation

 
   

Alpha-internexin (NF66)

 

cell differentiation, morphogenesis of neurons

 
   

Protein-tyrosine receptor type F polypeptide interacting protein (PTPRF)

 

cell adhesion receptor

 
    

Neuronal-specific enolase (NSE)

energy metabolism

 
   

Variation in ATP synthase, D chain

 

energy metabolism

 
   

Alpha-enolase-1 (ENO1)

 

glycolysis, growth control, hypoxia tolerance and allergic responses

 
   

Guanine nucleotide-binding proteins (G-proteins)

 

signal transduction

 

Cardiovascular system

      
 

Ischemia-induced cardiac injury (Kim N.)

2-DE, MALDI-TOF-MS

 

Prohibitin

cell cycle

Proteomic analysis provides appropriate means for identifying cardiac markers for detection of ischemia-induced cardiac injury.

    

VDAC

apoptosis

 
 

Contractile dysfunction (Essop MF.)

2D-PAGE, ESI-Q-TOF

 

ATP synthase D chain

OXPHOS

Decreased contractile protein levels may contribute to the contractile dysfunction of hearts from diabetic mice.

   

Ubiquinol cytochrome-C reductase core protein 1

 

electron transport

 
    

Electron transfer flavoprotein subunit α

electron transport

 

Liver disease

      
 

Acetaminophen (APAP) affected the liver (Ruepp SU.)

2D-DIGE, MALDI-TOF-MS

 

HSP10 and HSP60

protein assembly and folding

APAP toxicity was a direct action of its known reactive metabolite NAPQI, rather than a consequence of gene regulation.

   

Heat shock 70 kDa protein 9, (GRP75)

 

transporters and channels

 
 

High-fat diet induces hepatic steatosis (Eccleston HB.)

2D-IEF/SDS-PAGE

uMUP-VIII major urinary protein

 

pheromone communication (only in rodents)

HFD causes steatosis, alters NO metabolism, and modifies the liver mitochondrial proteome, thus, NO may play an important role in the processes responsible for NAFLD.

   

Thiosulfate sulfurtransferase

 

cyanide detoxification, role in iron-sulfur centers, sulfane metabolism

 
    

3-hydroxy-3-methylglutaryl-CoA synthase 2 (HMG-CoA synthase)

catalyzes the condensation of acetoacetyl CoA and acetone step in ketogenesis

 
    

Succinate dehydrogenase subunit a (SDH-A)

catalyzes the oxidation of succinate to fumarate, flavoprotein

 
    

ATP synthase F1 α and β subunits

OXPHOS

 

Skeletal muscl e

      
 

Hypoxia-induced changes in rat skeletal muscle (De Palma S.)

2D-DIGE, HPLC ESI-MS/MS

Hypoxia inducible factor 1 (HIF-1R)

 

transcription

In vivo adaptation to hypoxia requires an active metabolic switch.

   

Pyruvate dehydrogenase kinase 1 (PDK1)

 

regulation of glucose metabolism

 
    

Mitochondrial dihydrolipoamide dehydrogenase

branched chain family amino acid catabolic process

 
    

Succinyl CoA ligase α chain

tricarboxylic acid cycle

 
 

Lifestyle on the aging alterations ( Alves RM.)

2-DE, MALDI-TOF/TOF

NADH dehydrogenase [ubiquinone] 1 α subcomplex subunit 4

 

oxidative phosphorylation

Lifestyle is a key modulator for preventing aging-induced protein expression and functionality in mitochondria.

   

Creatine kinase

 

signal transduction

 
   

Superoxide dismutase [Mn], mitochondrial

 

redox

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