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Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants
The data is related to the research article entitled “Hypomyelinating leukodystrophy-associated missense mutation in HSPD1 blunts mitochondrial dynamics” [1]. In addition to hypomyelinating leukodystrophy (HLD) 4 (OMIM no. 612233), it is known that spastic paraplegia (SPG) 13 (OMIM no. 605280) is ca...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4716458/ https://www.ncbi.nlm.nih.gov/pubmed/26900593 http://dx.doi.org/10.1016/j.dib.2015.12.038 |
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author | Miyamoto, Yuki Megumi, Funakoshi-Tago Hasegawa, Nanami Eguchi, Takahiro Tanoue, Akito Tamura, Hiroomi Yamauchi, Junji |
author_facet | Miyamoto, Yuki Megumi, Funakoshi-Tago Hasegawa, Nanami Eguchi, Takahiro Tanoue, Akito Tamura, Hiroomi Yamauchi, Junji |
author_sort | Miyamoto, Yuki |
collection | PubMed |
description | The data is related to the research article entitled “Hypomyelinating leukodystrophy-associated missense mutation in HSPD1 blunts mitochondrial dynamics” [1]. In addition to hypomyelinating leukodystrophy (HLD) 4 (OMIM no. 612233), it is known that spastic paraplegia (SPG) 13 (OMIM no. 605280) is caused by HSPD1’s amino acid mutation. Two amino acid mutations Val-98-to-Ile (V98I) and Gln-461-to-Glu (Q461E) are associated with SPG13 [2]. In order to investigate the effects of HSPD1’s V98I or Q461E mutant on mitochondrial morphological changes, we transfected each of the respective mutant-encoding genes into Cos-7 cells. Either of V98I or Q461E mutant exhibited increased number of mitochondria and short length mitochondrial morphologies. Using MitoTracker dye-incorporating assay, decreased mitochondrial membrane potential was also observed in both cases. The data described here supports that SPG13-associated HSPD1 mutant participates in causing aberrant mitochondrial morphological changes with decreased activities. |
format | Online Article Text |
id | pubmed-4716458 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-47164582016-02-19 Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants Miyamoto, Yuki Megumi, Funakoshi-Tago Hasegawa, Nanami Eguchi, Takahiro Tanoue, Akito Tamura, Hiroomi Yamauchi, Junji Data Brief Data Article The data is related to the research article entitled “Hypomyelinating leukodystrophy-associated missense mutation in HSPD1 blunts mitochondrial dynamics” [1]. In addition to hypomyelinating leukodystrophy (HLD) 4 (OMIM no. 612233), it is known that spastic paraplegia (SPG) 13 (OMIM no. 605280) is caused by HSPD1’s amino acid mutation. Two amino acid mutations Val-98-to-Ile (V98I) and Gln-461-to-Glu (Q461E) are associated with SPG13 [2]. In order to investigate the effects of HSPD1’s V98I or Q461E mutant on mitochondrial morphological changes, we transfected each of the respective mutant-encoding genes into Cos-7 cells. Either of V98I or Q461E mutant exhibited increased number of mitochondria and short length mitochondrial morphologies. Using MitoTracker dye-incorporating assay, decreased mitochondrial membrane potential was also observed in both cases. The data described here supports that SPG13-associated HSPD1 mutant participates in causing aberrant mitochondrial morphological changes with decreased activities. Elsevier 2016-01-04 /pmc/articles/PMC4716458/ /pubmed/26900593 http://dx.doi.org/10.1016/j.dib.2015.12.038 Text en © 2016 The Authors http://creativecommons.org/licenses/by/4.0/ This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Data Article Miyamoto, Yuki Megumi, Funakoshi-Tago Hasegawa, Nanami Eguchi, Takahiro Tanoue, Akito Tamura, Hiroomi Yamauchi, Junji Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants |
title | Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants |
title_full | Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants |
title_fullStr | Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants |
title_full_unstemmed | Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants |
title_short | Data supporting mitochondrial morphological changes by SPG13-associated HSPD1 mutants |
title_sort | data supporting mitochondrial morphological changes by spg13-associated hspd1 mutants |
topic | Data Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4716458/ https://www.ncbi.nlm.nih.gov/pubmed/26900593 http://dx.doi.org/10.1016/j.dib.2015.12.038 |
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