Dataset Information

Mitochondrial structure and bioenergetics in Pseudoxanthoma elasticum

ABSTRACT: Aim of the present study was to integrate structural and bioenergenetic features to deeply investigate mitochondria of control and of PXE fibroblasts cultured in standard conditions, to explore their role in the development of the fibroblasts’ pathologic phenotype.

INSTRUMENT(S):

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Fibroblast

DISEASE(S): Pseudoxanthoma Elasticum

SUBMITTER: Francesco Demetrio Lofaro

LAB HEAD: Federica Boraldi

PROVIDER: PXD021647 | Pride | 2023-03-10

REPOSITORIES: Pride

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Dataset's files

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			Action	DRS
	20190624_MitoControl_A_01.raw	Raw
	20190624_MitoControl_A_02.raw	Raw
	20190624_MitoControl_B_01.raw	Raw
	20190624_MitoControl_B_02.raw	Raw
	20190624_MitoControl_C_01.raw	Raw

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Publications

Relationship Between Mitochondrial Structure and Bioenergetics in Pseudoxanthoma elasticum Dermal Fibroblasts.

Lofaro Francesco Demetrio FD Boraldi Federica F Garcia-Fernandez Maria M Estrella Lara L Valdivielso Pedro P Quaglino Daniela D

Frontiers in cell and developmental biology 20201217

Pseudoxanthoma elasticum (PXE) is a genetic disease considered as a paradigm of ectopic mineralization disorders, being characterized by multisystem clinical manifestations due to progressive calcification of skin, eyes, and the cardiovascular system, resembling an age-related phenotype. Although fibroblasts do not express the pathogenic ABCC6 gene, nevertheless these cells are still under investigation because they regulate connective tissue homeostasis, generating the "arena" whe ...[more]

PMID: 33392199

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Dataset Information

Mitochondrial structure and bioenergetics in Pseudoxanthoma elasticum

Dataset's files

Publications

Relationship Between Mitochondrial Structure and Bioenergetics in <i>Pseudoxanthoma elasticum</i> Dermal Fibroblasts.

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