Table 1 A list of mitochondrial candidates that bind to Drp1 in neuronal cells derived from HD patient-iPS cells and not in cells from normal subjects

From: ATAD3A oligomerization causes neurodegeneration by coupling mitochondrial fragmentation and bioenergetics defects

Function group

Uniprot ID

Protein name

Symbol

Spec count

Mitochondrial nucleiod assembly

Q9NVI7

ATPase family AAA domain-containing protein 3A

ATAD3A

16

 

Q96EY7

Pentatricopeptide repeat-containing protein 3, mitochondrial

PTCD3

16

 

P19388

DNA-directed RNA polymerases I, II, and III subunit RPABC1

POLR2E

12

 

Q96EY1

DnaJ homolog subfamily A member 3, mitochondrial

DNAJA3

9

 

Q00059

Transcription factor A, mitochondrial

TFAM

4

 

Q07021

Complement component 1 Q subcomponent-binding protein, mitochondrial

C1QBP

7

 

P55084

Trifunctional enzyme subunit beta, mitochondrial

HADHB

5

Energy production

O75947

ATP synthase subunit d, mitochondrial

ATP5H

13

 

P14618

Pyruvate kinase isozymes M1/M2

PKM2

12

 

P24539

ATP synthase subunit b, mitochondrial

ATP5F1

6

 

P36542

ATP synthase subunit gamma, mitochondrial

ATP5C1

5

 

P22695

Cytochrome b-c1 complex subunit 2, mitochondrial

UQCRC2

5

 

O75964

ATP synthase subunit g, mitochondrial

ATP5L

5

 

P56134

ATP synthase subunit f, mitochondrial

ATP5J2

4

Mitochondrial chaperone

P38646

Stress-70 protein, mitochondrial

HSPA9

36

 

P08107

Heat shock 70 kDa protein 1 A/1B

HSPA1A

19

 

P54652

Heat shock-related 70 kDa protein 2

HSPA2

14

 

P34931

Heat shock 70 kDa protein 1-like

HSPA1L

9

Mitochondrial metabolism

P36957

Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial

DLST

14

 

O14874

[3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial

BCKDK

11

 

P11182

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial

DBT

5

Protein kinase

P67775

Serine/threonine-protein phosphatase 2 A catalytic subunit alpha isoform

PPP2CA

11

 

P12532

Creatine kinase U-type, mitochondrial

CKMT1A

11

 

P28482

Mitogen-activated protein kinase 1

MAPK1

11

Mitochodnrial membrane integrity

P05141

ADP/ATP translocase 2

SLC25A5

11

 

P45880

Voltage-dependent anion-selective channel protein 2

VDAC2

6

Redox signaling

P30048

Thioredoxin-dependent peroxide reductase, mitochondrial

PRDX3

5

Apoptotic signaling

Q8WUZ0

B-cell CLL/lymphoma 7 protein family member C

BCL7C

4

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