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Proteomic mapping of fibroblasts from patients with HSP60 deficiency

ABSTRACT: Deficiency of the mitochondrial chaperone HSP60 has detrimental effects on the mitochondrial functions, and can in patient’s lead to hypomyelination disorder. The present study investigates the proteome in cultivated skin fibroblasts originating from two patients with HSP60 genetic variants and hypomyelination.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Skin, Fibroblast

SUBMITTER: Johan Palmfeldt

LAB HEAD: Johan Palmfeldt

PROVIDER: PXD048923 | Pride | 2025-09-11

REPOSITORIES: Pride

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Dataset's files

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			Action	DRS
	Y109-Cagla-Fibro-TMT-3-3of50.raw	Raw
	Y109-Cagla-Fibro-TMT-merge-for-real_pd3.mzML	Mzml
	Y109-Cagla-Fibro-TMT-merge-for-real_pd3.mzid.gz	Mzid
	Y223-FibroCag-TMT-3of50uL-1-2.raw	Raw
	Y224-FibroCag-TMT-3of50uL-1-2-excl.raw	Raw

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1 - 5 of 18

Publications

HSP60 chaperone deficiency disrupts the mitochondrial matrix proteome and dysregulates cholesterol synthesis.

Cömert Cagla C Kjær-Sørensen Kasper K Hansen Jakob J Carlsen Jasper J Just Jesper J Meaney Brandon F BF Østergaard Elsebet E Luo Yonglun Y Oxvig Claus C Schmidt-Laursen Lisbeth L Palmfeldt Johan J Fernandez-Guerra Paula P Bross Peter P

Molecular metabolism 20240814

<h4>Objective</h4>Mitochondrial proteostasis is critical for cellular function. The molecular chaperone HSP60 is essential for cell function and dysregulation of HSP60 expression has been implicated in cancer and diabetes. The few reported patients carrying HSP60 gene variants show neurodevelopmental delay and brain hypomyelination. Hsp60 interacts with more than 260 mitochondrial proteins but the mitochondrial proteins and functions affected by HSP60 deficiency are poorly characterized.<h4>Meth ...[more]

PMID: 39147275

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Proteomics effects of HSP60 deficiency in HEK cells, using an inducible transgenic HSP60 ATPase deficient mutant.

Project description:To investigate the immediate effect of HSP60 dysfunction on mitochondrial functions, we induced the expression of a dominant-negative ATPase-deficient HSP60 mutant (HSP60-D423A) in HEK293 cells using tetracycline . Incorporation of HSP60-D423A subunits into HSP60 heptamer rings leads to dysfunction of the chaperonin complex. We titrated tetracycline levels to have a biologically relevant cellular model that can be monitored over time. Expression of the HSP60-D432A protein resulted in significantly reduced cell counts at 72 hours induction compared to uninduced and HSP60-WT co-expressing cells. To study the effects of onset of HSP60 deficiency, we monitored the proteomics changes using TMT based proteomics.

2025-05-06 | PXD048918 | Pride

The proteomics effects of HSP60 chaperone deficiency in zebrafish larvae

Project description:To understand the effects of Hsp60 deficiency in developing vertebrates, we generated CRISPR/Cas9-mediated hspd1 knockout zebrafish lines by targeting exon 2 to induce a frameshift mutation. We selected an allele with a 56 base pair deletion inducing a frameshift mutation leading to loss of protein functions. We examined the proteome changes in zebrafish larvae at 5 days post fertilization (DPF). Wildtype control and hspd1-/- larvae at 5dpf, were analyzed by TMT and nanoLC-MS/MS based proteomcis. For this purpose, we studied five pools from each genotype, and each pool consisted of five larvae.

2024-09-27 | PXD048920 | Pride

Cytosolic Hsp60 orchestrates the survival and inflammatory responses of vascular smooth muscle cells in injured aortic vessels by regulating NF-κB-dependent gene expression

Project description:Pro-inflammatory response of VSMCs is triggered by endothelial damage and a causative step for thrombosis and neointimal thickening in the arterial vessels. Therefore, we investigate a role of cytosolic Hsp60 as a novel pro-inflammatory mediator in VSMCs. Hsp60 was detected in the cytosol of VSMCs. The selective depletion of cytosolic Hsp60 in VSMCs reduced the IKK activation, repressed the induction of NF-κB-dependent pro-survival genes (MnSOD and Bfl-1/A1), and enhanced apoptotic death in response to TNF-α. Moreover, a quantitative RNA sequencing revealed that the expression of 75 genes among the 774 TNF-α-inducible genes was significantly reduced by the depletion of cytosolic Hsp60. In particular, the expression of pro-inflammatory cytokines/chemokines, such as CCL2, CCL20, and IL-6, was regulated by the cytosolic Hsp60 in VSMCs. Finally, the depletion of cytosolic Hsp60 markedly inhibited the neointimal thickening in the balloon-injured arterial vessels by inducing apoptotic cell death and inhibiting chemokine production. This study provides the first evidence that cytosolic Hsp60 could be a therapeutic target for preventing inflammation-driven VSMC hyperplasia in the injured vessels. Hsp60 normal vs knockout with TNF-alpha treatment

2015-03-01 | E-GEOD-65354 | biostudies-arrayexpress

Microarray of neural progenitor cells from neonatal lung injury mice treated with placebo or umbilical-cord mesenchymal stromal cell extracellular vesicles or untreated, as well as age-matched control mice

Project description:The purpose of this experiment was to determine the transcriptional differences between neural progenitor cells from neonatal lung injury mice vs. control mice, as well as neonatal lung injury mice treated with umbilical-cord mesenchymal stromal cell extracellular vesicles vs. neonatal lung injury mice treated with placebo (PBS). Neural progenitor cells were isolated from the subventricular zone and hippocampus and cultured for 2 consecutive neurosphere assays. RNA was then extracted from the cells, and the microarray labelling, hybridization, and scanning was conducted by the Génome Québec Innovation Centre (Montréal, Canada).

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Modelling HSP60 deficiency using an inducible transgenic HSP60 ATPase deficient mutant

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Expression profiles of E. coli groESL(-) cells expressing human Hsp60(wt)/Hsp10 or Hsp60-(p.Val98Ile)/Hsp10 operons

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Strategic destabilization of K-MT attachments in elderly cells delays senescence

Project description:Chromosomal instability (CIN) refers to the rate at which cells are unable to properly segregate whole chromosomes, leading to aneuploidy. Using young to old-aged human dermal fibroblasts, we observed a dysfunction of the mitotic machinery arising with age that mildly perturbs chromosome segregation fidelity and contributes to the generation of fully senescent cells. Here, we found that elderly cells have an increased number of stable kinetochore-microtubule (k-MT) attachments and decreased efficiency in the correction of improper k-MT interactions. Importantly, chromosome mis-segregation rates in old-aged cells decreased upon both genetic and small molecule enhancement of MT-depolymerizing kinesin-13 activity. Notably, restored chromosome segregation accuracy inhibited the phenotypes of cellular senescence.

2020-02-27 | E-MTAB-8634 | biostudies-arrayexpress

Cytosolic Hsp60 orchestrates the survival and inflammatory responses of vascular smooth muscle cells in injured aortic vessels by regulating NF-κB-dependent gene expression

2015-03-01 | GSE65354 | GEO

Transcription profiling of E. coli groESL(-) cells expressing human Hsp60(wt)/Hsp10 or Hsp60-(p.Val98Ile)/Hsp10 operons

Project description:To identify regulatory responses that could give clues as to the cause(s) of the growth arrest observed in E. coli cells expressing mutant Hsp60-(p.Val98Ile) and Hsp10 versus cells expressing wild type Hsp60 and Hsp10, we performed microarray analyses. We reasoned that the decreased chaperonin activity of the Hsp60-(p.Val98Ile) protein would greatly impair or abolish folding of E. coli proteins that depend on chaperonin assistance. The resulting decreased levels of various activities may cause compensatory up-regulation or deregulation of genes. The transcriptomes of cells shifted to expression of the arabinose-inducible chaperonin operon with either the Hsp60-(p.Val98Ile) mutant or the wild type Hsp60 from the second plasmid were compared. RNA samples from three independent cultures expressing the mutant or two independent cultures expressing wild type Hsp60 were pooled, respectively. Experiment Overall Design: B178 (groELS)-deleted cells double-transformed with two plasmids harboring an IPTG-inducible operon with wild type human Hsp60 and Hsp10 and a second plasmid harboring an arabinose inducible operon with either Hsp60-(p.Val98Ile) and Hsp10 (mutant cells) or wild type Hsp60 and Hsp10 (wild type cells) were grown in dYT medium (21) supplemented with kanamycin (10 mg/L), ampicillin (100 mg/L) and IPTG (0.1mM) at 30Â°C in a shaking water bath. At OD536 â?? 0.1 bacteria were harvested by centrifugation at ambient temperature, resuspended in fresh medium, and 0.2% arabinose was added. Growth was continued at 30Â°C. After 10 hours 3ml samples were withdrawn and RNA was isolated. Experiment Overall Design: Two independent transformant clones of the wild type cells and three of the mutant cells were grown in this way. The RNA preps from the 2 wild type and the three mutant cells, respectively, were pooled, cDNA was synthesized, labelled and subjected to Affymetrix arrays.

2008-06-11 | E-GEOD-10974 | biostudies-arrayexpress

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Project description:The uploaded datasets contains raw data from our research: columns represent patients, rows represent meta data (patients response, type of sarcoma, etc ...) + the raw gene counts.

2022-09-01 | E-MTAB-12163 | biostudies-arrayexpress

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