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Protein Page:
ADCK3 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
ADCK3 May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain. Defects in ADCK3 are the cause of coenzyme Q10 deficiency, primary, type 4 (COQ10D4). An autosomal recessive disorder characterized by childhood-onset of cerebellar ataxia and exercise intolerance. Patient manifest gait ataxia and cerebellar atrophy with slow progression. Additional features include brisk tendon reflexes and Hoffmann sign, variable psychomotor retardation and variable seizures. Belongs to the protein kinase superfamily. ADCK protein kinase family. 4 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC 2.7.11.-; Kinase, protein; Protein kinase, Ser/Thr (non-receptor); Protein kinase, atypical; ATYPICAL group; ABC1 family; ABC1-A subfamily
Chromosomal Location of Human Ortholog: 1q42.13
Cellular Component: mitochondrion
Molecular Function: protein serine/threonine kinase activity; ATP binding
Biological Process: cell death; ubiquinone biosynthetic process; protein amino acid phosphorylation
Reference #:  Q8NI60 (UniProtKB)
Alt. Names/Synonyms: aarF domain-containing protein kinase 3; ADCK3; ARCA2; CABC1; Chaperone activity of bc1 complex-like, mitochondrial; chaperone, ABC1 activity of bc1 complex homolog (S. pombe); chaperone, ABC1 activity of bc1 complex like; Chaperone-ABC1-like; coenzyme Q8 homolog; COQ8; MGC4849; SCAR9
Gene Symbols: ADCK3
Molecular weight: 71,950 Da
Basal Isoelectric point: 6.51  Predict pI for various phosphorylation states
Select Structure to View Below

ADCK3

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 K194 RPENKQHKQTLSEHA
0 1 S236-p LAEVAKKsLRsEDPS
0 1 S239-p VAKKsLRsEDPSGKk
0 2 K246-ub sEDPSGKkAVLGSSP
0 1 K269 RIVRTLCKVRGAALK
0 1 K295-ub FINPHLAkIFERVRQ
0 1 K310-ub SADFMPLkQMMkTLN
0 2 K314-ub MPLkQMMkTLNNDLG
0 1 K327 LGPNWRDKLEYFEER
0 2 K327-ub LGPNWRDkLEYFEER
0 1 Y408-p ELALECDyQREAACA
0 1 S517 ATREYDRSFTDLYIQ
0 2 A539 RDRETVRAKSIEMKF
0 1 K642-ub EAYSNYCkRQAQQ__
  mouse

 
K191-ub RPESKPHkQMLSERA
S233 LAEVAKKSLRSENST
S236 VAKKSLRSENSTGKk
K243-ub SENSTGKkAVLDSSP
K266-ub RIVSTLCkVRGAALK
K292 FINPHLAKIFERVRQ
K307 SADFMPLKQMTkTLN
K311-ub MPLKQMTkTLNSDLG
K324-ac LGPHWRDkLEYFEER
K324-ub LGPHWRDkLEYFEER
Y405 ELTLECDYQREAAYA
S514-p ATREYDRsFTDLYIQ
K536-ac QDREAVLkKSIEMKF
R639 EAYSNYCRMKSGLQ_
  rat

 
K195 RPESKPHKQMLSERA
S237 LAEVAKKSLRSENST
S240 VAKKSLRSENSTGKK
K247 SENSTGKKAVLDSSP
K270 RIVSTLCKVRGAALK
K296 FINPHLAKIFERVRQ
K311 SADFMPLKQMTKTLN
K315 MPLKQMTKTLNNDLG
K328 LGPHWRDKLEYFEER
K328 LGPHWRDKLEYFEER
Y409 ELTLECDYQREAAYA
S518 ATREYDRSFTDLYIQ
K540 QDREAVLKKSIEMKF
- under review  
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