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TitleCryoET reveals organelle phenotypes in huntington disease patient iPSC-derived and mouse primary neurons.
Journal, issue, pagesNat Commun, Vol. 14, Issue 1, Page 692, Year 2023
Publish dateFeb 8, 2023
AuthorsGong-Her Wu / Charlene Smith-Geater / Jesús G Galaz-Montoya / Yingli Gu / Sanket R Gupte / Ranen Aviner / Patrick G Mitchell / Joy Hsu / Ricardo Miramontes / Keona Q Wang / Nicolette R Geller / Cathy Hou / Cristina Danita / Lydia-Marie Joubert / Michael F Schmid / Serena Yeung / Judith Frydman / William Mobley / Chengbiao Wu / Leslie M Thompson / Wah Chiu /
PubMed AbstractHuntington'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 ...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, and wild-type mice. In HD models, we discovered sheet aggregates in double membrane-bound organelles, and mitochondria with distorted cristae and enlarged granules, likely mitochondrial RNA granules. We used artificial intelligence to quantify mitochondrial granules, and proteomics experiments reveal differential protein content in isolated HD mitochondria. Knockdown of Protein Inhibitor of Activated STAT1 ameliorated aberrant phenotypes in iPSC- and BACHD neurons. We show that integrated ultrastructural and proteomic approaches may uncover early HD phenotypes to accelerate diagnostics and the development of targeted therapeutics for HD.
External linksNat Commun / PubMed:36754966 / PubMed Central
MethodsEM (tomography)
Structure data

EMDB-28668: CryoET tomogram of iPSC-derived control non-HD neuron (Q18)
Method: EM (tomography)

EMDB-28944: CryoET tomogram of iPSC-derived control non-HD neuron (Q20)
Method: EM (tomography)

EMDB-28946: CryoET tomogram of HD patient iPSC-derived neuron (Q53)
Method: EM (tomography)

EMDB-29074: CryoET tomogram of HD patient iPSC-derived neuron (Q66)
Method: EM (tomography)

EMDB-29075: CryoET tomogram of HD patient iPSC-derived neuron (Q77)
Method: EM (tomography)

EMDB-29076: CryoET tomogram of HD patient iPSC-derived neuron (Q109)
Method: EM (tomography)

EMDB-29079: CryoET tomogram of HD patient iPSC-derived neuron (Q66) showing sheet aggregate
Method: EM (tomography)

EMDB-29080: CryoET tomogram of HD patient iPSC-derived neuron (Q66) with PIAS1 hetKO treatment
Method: EM (tomography)

EMDB-29081: CryoET tomogram of HD patient iPSC-derived neuron (Q66) with GRSF1 KD treatment
Method: EM (tomography)

EMDB-29083: CryoET tomogram of mitochondria in WT mouse neuron
Method: EM (tomography)

EMDB-29084: CryoET tomogram of mitochondria in BACHD-dN17 mouse model neuron
Method: EM (tomography)

EMDB-29207: CryoET tomogram of mitochondria in BACHD mouse model neuron
Method: EM (tomography)

EMDB-29208: CryoET tomogram of BACHD mouse model neuron showing sheet aggregates
Method: EM (tomography)

EMDB-29210: CryoET tomogram of purified mitochondria from HD patient iPSC-derived neuron (Q109)
Method: EM (tomography)

EMDB-29211: CryoET tomogram of HD patient iPSC-derived neuron (Q66) with PIAS1 hetKO treatment
Method: EM (tomography)

Source
  • Homo sapiens (human)
  • Muridae (mammal)

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