Caseinolytic peptidase B protein homolog (CLPB), also known as Skd3, is a mitochondrial AAA ATPase chaperone that in humans is encoded by the gene CLPB,[5][6][7] which encodes an adenosine triphosphate-(ATP) dependent chaperone. Skd3 is localized in mitochondria and widely expressed in human tissues. High expression in adult brain and low expression in granulocyte is found.[8][9] It is a potent protein disaggregase that chaperones the mitochondrial intermembrane space.[10] Mutations in the CLPB gene could cause autosomal recessive metabolic disorder with intellectual disability/developmental delay, congenital neutropenia, progressive brain atrophy, movement disorder, cataracts, and 3-methylglutaconic aciduria.[8][11] Recently, heterozygous, dominant negative mutations in CLPB have been identified as a cause of severe congenital neutropenia (SCN).[12]
^ abcGRCh38: Ensembl release 89: ENSG00000162129 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000001829 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^Wiemann S, Weil B, Wellenreuther R, Gassenhuber J, Glassl S, Ansorge W, Böcher M, Blöcker H, Bauersachs S, Blum H, Lauber J, Düsterhöft A, Beyer A, Köhrer K, Strack N, Mewes HW, Ottenwälder B, Obermaier B, Tampe J, Heubner D, Wambutt R, Korn B, Klein M, Poustka A (March 2001). "Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs". Genome Research. 11 (3): 422–35. doi:10.1101/gr.GR1547R. PMC 311072. PMID 11230166.
^Périer F, Radeke CM, Raab-Graham KF, Vandenberg CA (January 1995). "Expression of a putative ATPase suppresses the growth defect of a yeast potassium transport mutant: identification of a mammalian member of the Clp/HSP104 family". Gene. 152 (2): 157–63. doi:10.1016/0378-1119(94)00697-Q. PMID 7835694.
^"Entrez Gene: CLPB ClpB caseinolytic peptidase B homolog (E. coli)".
^ abWortmann SB, Ziętkiewicz S, Kousi M, Szklarczyk R, Haack TB, Gersting SW, et al. (February 2015). "CLPB mutations cause 3-methylglutaconic aciduria, progressive brain atrophy, intellectual disability, congenital neutropenia, cataracts, movement disorder". American Journal of Human Genetics. 96 (2): 245–57. doi:10.1016/j.ajhg.2014.12.013. PMC 4320260. PMID 25597510.
^Saunders C, Smith L, Wibrand F, Ravn K, Bross P, Thiffault I, Christensen M, Atherton A, Farrow E, Miller N, Kingsmore SF, Ostergaard E (February 2015). "CLPB variants associated with autosomal-recessive mitochondrial disorder with cataract, neutropenia, epilepsy, and methylglutaconic aciduria". American Journal of Human Genetics. 96 (2): 258–65. doi:10.1016/j.ajhg.2014.12.020. PMC 4320254. PMID 25597511.
^Cupo, Ryan R; Shorter, James (2020-06-23). Berger, James M (ed.). "Skd3 (human CLPB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations". eLife. 9: e55279. doi:10.7554/eLife.55279. ISSN 2050-084X. PMC 7343390. PMID 32573439.
^Kiykim A, Garncarz W, Karakoc-Aydiner E, Ozen A, Kiykim E, Yesil G, Boztug K, Baris S (April 2016). "Novel CLPB mutation in a patient with 3-methylglutaconic aciduria causing severe neurological involvement and congenital neutropenia". Clinical Immunology. 165: 1–3. doi:10.1016/j.clim.2016.02.008. PMID 26916670.
^Warren, Julia T; Cupo, Ryan R; Wattanasirakul, Peeradol; Spencer, David; Locke, Adam E; Makaryan, Vahagn; Bolyard, Audrey Anna; Kelley, Meredith L; Kingston, Natalie L; Shorter, James; Bellanné-Chantelot, Christine (2021-06-11). "Heterozygous Variants of CLPB are a Cause of Severe Congenital Neutropenia". Blood. 139 (blood.2021010762): 779–791. doi:10.1182/blood.2021010762. ISSN 0006-4971. PMC 8814677. PMID 34115842.
B protein homolog (CLPB), also known as Skd3, is a mitochondrial AAA ATPase chaperone that in humans is encoded by the gene CLPB, which encodes an adenosine...
α-synuclein, TDP-43, FUS, and TAF15 in yeast cells. Conserved in prokaryotes (ClpB), fungi, plants and as well as animal mitochondria, there is yet to see hsp104...
Maintenance of steroid receptors and transcription factors 100 kDa ClpB, ClpA, ClpX Hsp104 (CLPB) Unfolding of insoluble protein aggregates; co-factor of DnaK/Hsp70...
signature indel has also been identified; this three-amino-acid deletion in ClpB chaperone is present in all species of the Bacteroidota phylum except S....
associated with a protease and have functions other than proteolysis. ClpB (human CLPB "Hsp78", yeast Hsp104) break up insoluble protein aggregates in conjunction...
alpha-MSH are, in fact, generated against ClpB, a protein produced by certain gut bacteria e.g. Escherichia coli. ClpB protein was identified as a conformational...
(DnaK-DnaJ-GrpE in E. coli and Ssa1-Ydj1/Sis1-Sse1/Fe1 in yeast) and Hsp100 (ClpB in E. coli and Hsp104 in yeast) chaperones for protein disaggregation and...
uniquely activate macrophages in response to infection. The bacterial chaperone ClpB is a major driver in the overall virulence of L. interrogans, as it aids...
PMID 1094538. Squires, C L; Pedersen, S; Ross, B M; Squires, C (1991). "ClpB is the Escherichia coli heat shock protein F84.1". Journal of Bacteriology...
into structural and functional relationships between LonA proteases and ClpB chaperones". FEBS Open Bio. 9 (9): 1536–1551. doi:10.1002/2211-5463.12691...
Proteins IbpA and IbpB in Thermal Stress Management: Comparison with ClpA, ClpB, and HtpG In Vivo". J. Bacteriol. 180 (19): 5165–72. doi:10.1128/JB.180.19...
secretion system (T4SS) genes such as traG and improved by the addition of the clpB chaperone gene. The invasion of legume by Mesorhizobium mediterraneum was...
ISSN 1043-4666. PMID 11554782. Cupo RR, Shorter J (2020-06-23). "Skd3 (human CLPB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic...
identity. The predicted structure is Chain A, crystal structure analysis of Clpb, a protein that encodes an ATP-dependent protease and chaperone. This protein...
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