Results 111 to 120 of about 18,329 (223)

Elevated Plasma Phospho‐Tau217 in Beta‐Propeller Protein‐Associated Neurodegeneration

Movement Disorders, EarlyView.
Jae‐Hyeok Lee, Yun Soo Kim
wiley   +1 more source

Canonical and non‐canonical functions of proteins regulating mitochondrial dynamics in mammalian physiology

The Journal of Physiology, EarlyView.
Abstract figure legend Mitochondria are highly dynamic organelles that continuously remodel their architecture through coordinated cycles of fusion and fission. This review examines the four key GTPases that orchestrate mitochondrial dynamics in mammals: MFN1, MFN2, OPA1, and DRP1.
Rémi Chaney, Vincent Marcangeli, Krystel Desjardins, Shima Taherkhani, Gilles Gouspillou   +4 more
wiley   +1 more source

Mitochondrial iron deficiency triggers cytosolic iron overload in PKAN hiPS-derived astrocytes

Cell Death and Disease
Disease models of neurodegeneration with brain iron accumulation (NBIA) offer the possibility to explore the relationship between iron dyshomeostasis and neurodegeneration. We analyzed hiPS-derived astrocytes from PANK2-associated neurodegeneration (PKAN)
Paolo Santambrogio, Anna Cozzi, Chiara Balestrucci, Maddalena Ripamonti, Valeria Berno, Eugenia Cammarota, Andrea Stefano Moro, Sonia Levi   +7 more
doaj   +1 more source

Towards Precision Therapies for Inherited Disorders of Neurodegeneration with Brain Iron Accumulation. [PDF]

Tremor Other Hyperkinet Mov (N Y), 2021
Spaull RVV, Soo AKS, Hogarth P, Hayflick SJ, Kurian MA.   +4 more
europepmc   +1 more source

Metabolic impairments in neurodegeneration with brain iron accumulation

Biochimica et Biophysica Acta (BBA) - Bioenergetics
Neurodegeneration with brain iron accumulation (NBIA) is a broad, heterogeneous group of rare inherited diseases (1-3 patients/1,000,000 people) characterized by progressive symptoms associated with excessive abnormal iron deposition in the brain. Approximately 15,000-20,000 individuals worldwide are estimated to be affected by NBIA.
Agata, Wydrych, Barbara, Pakuła, Justyna, Janikiewicz, Aneta M, Dobosz, Patrycja, Jakubek-Olszewska, Marta, Skowrońska, Iwona, Kurkowska-Jastrzębska, Maciej, Cwyl, Mariola, Popielarz, Paolo, Pinton, Barbara, Zavan, Agnieszka, Dobrzyń, Magdalena, Lebiedzińska-Arciszewska, Mariusz R, Więckowski   +13 more
openaire   +2 more sources

Advances in the Neurotoxic Mechanisms of Acrylamide and Multitarget Intervention Strategies of Natural Products

Food Frontiers, Volume 7, Issue 4, July 2026.
This review summarizes recent advances in acrylamide neurotoxicity mechanisms and highlights the multitarget neuroprotective effects of diverse natural products while also addressing current challenges and future translational research directions.
Wen Du, Yifan Zhao, Li Dong, Chen Ma, Xiaosong Hu, Daotong Li, Fang Chen, Yinghua Luo   +7 more
wiley   +1 more source

Disruption of intracellular iron homeostasis through mitochondrial dysfunction associated with suppression of ATP 13A2 expression

Scientific Reports
Elevated iron in the SNpc may play a key role in Parkinson’s disease (PD) neurodegeneration, yet the underlying mechanism accounting for this iron accumulation is unclear. Although iron is an essential element, excessive amounts produce toxicity.
Takanori Murakami, Kazuki Ohuchi, Masanori Kiuchi, Hisaka Kurita, Kanta Kawai, Ryo Kakiuchi, Tasuku Hirayama, Hideko Nagasawa, Zhiliang Wu, Yoichi Maekawa, Isao Hozumi, Masatoshi Inden   +11 more
doaj   +1 more source

Lifetime risk of autosomal recessive neurodegeneration with brain iron accumulation (NBIA) disorders calculated from genetic databases. [PDF]

EBioMedicine, 2022
Kolarova H, Tan J, Strom TM, Meitinger T, Wagner M, Klopstock T.   +5 more
europepmc   +1 more source

Ischemia–Reperfusion Injury: Molecular Mechanisms and Therapeutic Interventions

MedComm, Volume 7, Issue 7, July 2026.
Multiorgan ischemia–reperfusion injury begins with ischemia‐induced ATP depletion and ionic imbalance, followed by reperfusion‐triggered mitochondrial ROS/RNS bursts, regulated cell death, and DAMP release. Sterile inflammation converges on endothelial–immune–coagulation crosstalk, where NETs drive immunothrombosis, no‐reflow, and remote organ injury ...
Peng An, Yi An, Mengwei Chen, Longlong Wu, Rong Wang   +4 more
wiley   +1 more source

C19orf12 mutations in neurodegeneration with brain iron accumulation mimicking juvenile amyotrophic lateral sclerosis

, 2012
Mutations in C19orf12 have been recently identified as the molecular genetic cause of a subtype of neurodegeneration with brain iron accumulation (NBIA).
Prokisch, H., Haack, T.B., Behrmann, C., Gaul, C., Deschauer, M., Zierz, S.   +5 more
core   +2 more sources