Property Summary

NCBI Gene PubMed Count 19
Grant Count 59
R01 Count 28
Funding $5,573,552.96
PubMed Score 295.22
PubTator Score 73.18

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (3)

Disease log2 FC p
osteosarcoma -1.669 0.000
lung cancer 1.400 0.001
ovarian cancer 1.100 0.000

Synonym

Accession Q9H078 B4DXJ7 B4DXP7 B4DXW4 E7EWN6 F8W7P6 Q8ND11 Q9H8Y0
Symbols SKD3
HSP78
MGCA7
ANKCLB
MEGCANN

Gene

PANTHER Protein Class (2)

Gene RIF (8)

PMID Text
25650066 Disruption of CLPB is associated with congenital microcephaly, severe encephalopathy and 3-methylglutaconic aciduria
25597511 CLPB is proposed to function as a mitochondrial chaperone involved in disaggregation of misfolded proteins, resulting from stress such as heat denaturation.
25597510 Mutations in CLPB define a syndrome with intellectual disability, congenital neutropenia, progressive brain atrophy, movement disorder, cataracts, and 3-methylglutaconic aciduria.
25595726 Case Reports: bi-allelic CLPB mutations cause cataract, renal cysts, nephrocalcinosis and 3-methylglutaconic aciduria, a novel disorder of mitochondrial protein disaggregation.
25288401 ClpB can passively thread soluble denatured proteins.
20877624 Observational study of gene-disease association. (HuGE Navigator)
19698713 formation of the DnaK-ClpB bichaperone network is a three step process
18488042 ClpB-DnaK reactivated all aggregated fusion proteins with similar efficiency, without unfolding native domains, demonstrating that partial threading of the misfolded moiety is sufficient to solubilize aggregates.

AA Sequence

MLGSLVLRRKALAPRLLLRLLRSPTLRGHGGASGRNVTTGSLGEPQWLRVATGGRPGTSPALFSGRGAAT      1 - 70
GGRQGGRFDTKCLAAATWGRLPGPEETLPGQDSWNGVPSRAGLGMCALAAALVVHCYSKSPSNKDAALLE     71 - 140
AARANNMQEVSRLLSEGADVNAKHRLGWTALMVAAINRNNSVVQVLLAAGADPNLGDDFSSVYKTAKEQG    141 - 210
IHSLEDGGQDGASRHITNQWTSALEFRRWLGLPAGVLITREDDFNNRLNNRASFKGCTALHYAVLADDYR    211 - 280
TVKELLDGGANPLQRNEMGHTPLDYAREGEVMKLLRTSEAKYQEKQRKREAEERRRFPLEQRLKEHIIGQ    281 - 350
ESAIATVGAAIRRKENGWYDEEHPLVFLFLGSSGIGKTELAKQTAKYMHKDAKKGFIRLDMSEFQERHEV    351 - 420
AKFIGSPPGYVGHEEGGQLTKKLKQCPNAVVLFDEVDKAHPDVLTIMLQLFDEGRLTDGKGKTIDCKDAI    421 - 490
FIMTSNVASDEIAQHALQLRQEALEMSRNRIAENLGDVQISDKITISKNFKENVIRPILKAHFRRDEFLG    491 - 560
RINEIVYFLPFCHSELIQLVNKELNFWAKRAKQRHNITLLWDREVADVLVDGYNVHYGARSIKHEVERRV    561 - 630
VNQLAAAYEQDLLPGGCTLRITVEDSDKQLLKSPELPSPQAEKRLPKLRLEIIDKDSKTRRLDIRAPLHP    631 - 700
EKVCNTI                                                                   701 - 707
//

Text Mined References (24)

PMID Year Title
25944712 2015 N-terminome analysis of the human mitochondrial proteome.
25710177 2015 Metabolic and chaperone gene loss marks the origin of animals: evidence for Hsp104 and Hsp78 chaperones sharing mitochondrial enzymes as clients.
25650066 2015 Disruption of CLPB is associated with congenital microcephaly, severe encephalopathy and 3-methylglutaconic aciduria.
25597511 2015 CLPB variants associated with autosomal-recessive mitochondrial disorder with cataract, neutropenia, epilepsy, and methylglutaconic aciduria.
25597510 2015 CLPB mutations cause 3-methylglutaconic aciduria, progressive brain atrophy, intellectual disability, congenital neutropenia, cataracts, movement disorder.
25595726 2015 Bi-allelic CLPB mutations cause cataract, renal cysts, nephrocalcinosis and 3-methylglutaconic aciduria, a novel disorder of mitochondrial protein disaggregation.
25416956 2014 A proteome-scale map of the human interactome network.
25288401 2014 ClpB chaperone passively threads soluble denatured proteins through its central pore.
24275569 2014 An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
21269460 2011 Initial characterization of the human central proteome.
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