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Proteomic analysis of mitochondria from Huntington's Disease patient iPSC-derived neurons

ABSTRACT: Huntington's disease is caused by an expanded CAG repeat in the huntingtin gene, yeilding a Huntingtin protein with an expanded polyglutamine tract. Patient-derived induced pluripotent stem cells (iPSCs) can help understand disease; however, defining pathological biomarkers in challanging. Here we used LC-MS/MS to determine differences in mitochondrial proteome between iPSC-derived neurons from healthy donors and Huntington's disease patients.

INSTRUMENT(S): LTQ Orbitrap Elite

ORGANISM(S): Homo Sapiens (human)

TISSUE(S): Cell Culture

SUBMITTER: Ranen Aviner

LAB HEAD: Ranen Aviner

PROVIDER: PXD037526 | Pride | 2023-02-10

REPOSITORIES: Pride

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			Action	DRS
	P20210603-03_WT_1.raw	Raw
	P20210603-04_WT_2.raw	Raw
	P20210603-05_WT_3.raw	Raw
	P20210603-11_HD_1.raw	Raw
	P20210603-12_HD_2.raw	Raw

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Publications

CryoET reveals organelle phenotypes in huntington disease patient iPSC-derived and mouse primary neurons.

Wu Gong-Her GH Smith-Geater Charlene C Galaz-Montoya Jesús G JG Gu Yingli Y Gupte Sanket R SR Aviner Ranen R Mitchell Patrick G PG Hsu Joy J Miramontes Ricardo R Wang Keona Q KQ Geller Nicolette R NR Hou Cathy C Danita Cristina C Joubert Lydia-Marie LM Schmid Michael F MF Yeung Serena S Frydman Judith J Mobley William W Wu Chengbiao C Thompson Leslie M LM Chiu Wah W

Nature communications 20230208 1

Huntington's disease (HD) is caused by an expanded CAG repeat in the huntingtin gene, yielding a Huntingtin protein with an expanded polyglutamine tract. While experiments with patient-derived induced pluripotent stem cells (iPSCs) can help understand disease, defining pathological biomarkers remains challenging. Here, we used cryogenic electron tomography to visualize neurites in HD patient iPSC-derived neurons with varying CAG repeats, and primary cortical neurons from BACHD, deltaN17-BACHD, a ...[more]

PMID: 36754966

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Transcriptome profiling in knock-in mouse models of Huntington's disease [Striatum_miRNA]

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Project description:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that is characterized by motor, cognitive, and psychiatric alterations. The mutation responsible for this disease is an abnormally expanded and unstable CAG repeat within the coding region of the gene encoding huntingtin (Htt). Knock-in mouse models of HD with human exon 1 containing expanded CAG repeats inserted in the murine huntingtin gene (Hdh) provide a genetic reconstruction of the human causative mutation within the mouse model. The goal of this study is RNA expression profiling by RNA sequencing (RNA-seq) in 6 and 10 months old knock-in mice with CAG lengths of 20, 50, 92, 140 along with littermate control wild-type animals

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Project description:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that is characterized by motor, cognitive, and psychiatric alterations. The mutation responsible for this disease is an abnormally expanded and unstable CAG repeat within the coding region of the gene encoding huntingtin (Htt). Knock-in mouse models of HD with human exon 1 containing expanded CAG repeats inserted in the murine huntingtin gene (Hdh) provide a genetic reconstruction of the human causative mutation within the mouse model. The goal of this study is RNA expression profiling by RNA sequencing (RNA-seq) in 6 and 10 month old knock-in mice with CAG lengths of 20, 50, 92, 140 along with littermate control wild-type animals

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Project description:Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that is characterized by motor, cognitive, and psychiatric alterations. The mutation responsible for this disease is an abnormally expanded and unstable CAG repeat within the coding region of the gene encoding huntingtin (Htt). Knock-in mouse models of HD with human exon 1 containing expanded CAG repeats inserted in the murine huntingtin gene (Hdh) provide a genetic reconstruction of the human causative mutation within the mouse model. The goal of this study is RNA expression profiling by RNA sequencing (RNA-seq) in 2, 6, and 10 month old knock-in mice with CAG lengths of 20, 80, 92, 111, 140, 175 along with littermate control wild-type animals.

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