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Article ; Online: Mitochondria in neurodegeneration.

Chu, Charleen T

2022 Volume 26

Abstract: The brain is one of the most energetically demanding tissues in the human body, and mitochondrial pathology is strongly implicated in chronic neurodegenerative diseases. In contrast to acute brain injuries in which bioenergetics and cell death play ... ...

Abstract	The brain is one of the most energetically demanding tissues in the human body, and mitochondrial pathology is strongly implicated in chronic neurodegenerative diseases. In contrast to acute brain injuries in which bioenergetics and cell death play dominant roles, studies modeling familial neurodegeneration implicate a more complex and nuanced relationship involving the entire mitochondrial life cycle. Recent literature on mitochondrial mechanisms in Parkinson's disease, Alzheimer's disease, frontotemporal dementia, Huntington's disease, and amyotrophic lateral sclerosis is reviewed with an emphasis on mitochondrial quality control, transport and synaptodendritic calcium homeostasis. Potential neuroprotective interventions include targeting the mitochondrial kinase PTEN-induced kinase 1 (PINK1), which plays a role in regulating not only multiple facets of mitochondrial biology, but also neuronal morphogenesis and dendritic arborization.
Language	English
Publishing date	2022-04-01
Publishing country	England
Document type	Journal Article
ZDB-ID	2918626-2
ISSN	2468-8673 ; 2468-8681
ISSN (online)	2468-8673
ISSN	2468-8681
DOI	10.1016/j.cophys.2022.100532
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Autophagy in neurological diseases: An update.

Chu, Charleen T

Neurobiology of disease

2018 Volume 122, Page(s) 1–2

MeSH term(s)	Animals ; Autophagy ; Humans ; Nervous System Diseases/genetics ; Nervous System Diseases/physiopathology ; Nervous System Diseases/therapy
Language	English
Publishing date	2018-10-18
Publishing country	United States
Document type	Editorial ; Introductory Journal Article
ZDB-ID	1211786-9
ISSN	1095-953X ; 0969-9961
ISSN (online)	1095-953X
ISSN	0969-9961
DOI	10.1016/j.nbd.2018.10.010
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Zs.A 4444: Show issues

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Article ; Online: PINK1: Multiple mechanisms of neuroprotection.

Lizama, Britney N / Otero, P Anthony / Chu, Charleen T

International review of movement disorders

2021 Volume 2, Page(s) 193–219

Language	English
Publishing date	2021-10-04
Publishing country	England
Document type	Journal Article
ISSN	2666-7878
ISSN (online)	2666-7878
DOI	10.1016/bs.irmvd.2021.08.003
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Article ; Online: Neuronal autophagy and mitophagy in Parkinson's disease.

Lizama, Britney N / Chu, Charleen T

Molecular aspects of medicine

2021 Volume 82, Page(s) 100972

Abstract: Autophagy is the process by which cells can selectively or non-selectively remove damaged proteins and organelles. As the cell's main means of sequestering damaged mitochondria for removal, mitophagy is central to cellular function and survival. Research ...

Abstract	Autophagy is the process by which cells can selectively or non-selectively remove damaged proteins and organelles. As the cell's main means of sequestering damaged mitochondria for removal, mitophagy is central to cellular function and survival. Research on autophagy and mitochondrial quality control has increased exponentially in relation to the pathogenesis of numerous disease conditions, from cancer and immune diseases to chronic neurodegenerative diseases like Parkinson's disease (PD). Understanding how components of the autophagic/mitophagic machinery are affected during disease, as well as the contextual relationship of autophagy with determining neuronal health and function, is essential to the goal of designing therapies for human disease. In this review, we will summarize key signaling molecules that consign damaged mitochondria for autophagic degradation, describe the relationship of genes linked to PD to autophagy/mitophagy dysfunction, and discuss additional roles of both mitochondrial and cytosolic pools of PTEN-induced kinase 1 (PINK1) in mitochondrial homeostasis, dendritic morphogenesis and inflammation.
MeSH term(s)	Autophagy ; Humans ; Mitochondria ; Mitophagy ; Neurons ; Parkinson Disease/genetics ; Protein Kinases
Chemical Substances	Protein Kinases (EC 2.7.-) ; PTEN-induced putative kinase (EC 2.7.11.1)
Language	English
Publishing date	2021-06-12
Publishing country	England
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Review
ZDB-ID	197640-0
ISSN	1872-9452 ; 0098-2997
ISSN (online)	1872-9452
ISSN	0098-2997
DOI	10.1016/j.mam.2021.100972
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Zs.A 1189: Show issues

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Article ; Online: Mechanisms of selective autophagy and mitophagy: Implications for neurodegenerative diseases.

Chu, Charleen T

Neurobiology of disease

2018 Volume 122, Page(s) 23–34

Abstract: Over the past 20 years, the concept of mammalian autophagy as a nonselective degradation system has been repudiated, due in part to important discoveries in neurodegenerative diseases, which opened the field of selective autophagy. Protein aggregates and ...

Abstract	Over the past 20 years, the concept of mammalian autophagy as a nonselective degradation system has been repudiated, due in part to important discoveries in neurodegenerative diseases, which opened the field of selective autophagy. Protein aggregates and damaged mitochondria represent key pathological hallmarks shared by most neurodegenerative diseases. The landmark discovery in 2007 of p62/SQSTM1 as the first mammalian selective autophagy receptor defined a new family of autophagy-related proteins that serve to target protein aggregates, mitochondria, intracellular pathogens and other cargoes to the core autophagy machinery via an LC3-interacting region (LIR)-motif. Notably, mutations in the LIR-motif proteins p62 (SQSTM1) and optineurin (OPTN) contribute to familial forms of frontotemporal dementia and amyotrophic lateral sclerosis. Moreover, a subset of LIR-motif proteins is involved in selective mitochondrial degradation initiated by two recessive familial Parkinson's disease genes. PTEN-induced kinase 1 (PINK1) activates the E3 ubiquitin ligase Parkin (PARK2) to mark depolarized mitochondria for degradation. An extensive body of literature delineates key mechanisms in this pathway, based mostly on work in transformed cell lines. However, the potential role of PINK1-triggered mitophagy in neurodegeneration remains a conundrum, particularly in light of recent in vivo mitophagy studies. There are at least three major mechanisms by which mitochondria are targeted for mitophagy: transmembrane receptor-mediated, ubiquitin-mediated and cardiolipin-mediated. This review summarizes key features of the major cargo recognition pathways for selective autophagy and mitophagy, highlighting their potential impact in the pathogenesis or amelioration of neurodegenerative diseases.
MeSH term(s)	Animals ; Autophagy/physiology ; Humans ; Mitophagy/physiology ; Neurodegenerative Diseases/metabolism
Language	English
Publishing date	2018-07-17
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	1211786-9
ISSN	1095-953X ; 0969-9961
ISSN (online)	1095-953X
ISSN	0969-9961
DOI	10.1016/j.nbd.2018.07.015
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Article ; Online: Multiple pathways for mitophagy: A neurodegenerative conundrum for Parkinson's disease.

Chu, Charleen T

Neuroscience letters

2018 Volume 697, Page(s) 66–71

Abstract: It has been nearly a decade since the first landmark studies implicating familial recessive Parkinson's disease genes in the regulation of selective mitochondrial autophagy. The PTEN-induced kinase 1 (PINK1) and the E3 ubiquitin ligase Parkin (encoded by ...

Abstract	It has been nearly a decade since the first landmark studies implicating familial recessive Parkinson's disease genes in the regulation of selective mitochondrial autophagy. The PTEN-induced kinase 1 (PINK1) and the E3 ubiquitin ligase Parkin (encoded by the PARK2 gene) act together to mark depolarized mitochondria for degradation. There is now an extensive body of literature detailing key mediators and steps in this pathway, based mostly on work in transformed cell lines. However, the degree to which PINK1-triggered mitophagy contributes to mitochondrial quality control in the mammalian brain, and the extent to which its disruption contributes to Parkinson's disease pathogenesis remain uncertain. In recent years, it has become clear that there are multiple, potentially redundant, pathways of cargo specification for mitophagy. Important mitophagy-independent functions of PINK1 and Parkin are also emerging. This review summarizes key features of three major mitophagy cargo recognition systems: receptor-mediated, ubiquitin-mediated and cardiolipin-mediated. New animal models that may be useful for tracking the delivery of mitochondria into lysosomes in different neuronal populations will be highlighted. Combining these research tools with methods to selectively disrupt specific mitophagy pathways may lead to a better understanding of the potential role of mitophagy in modulating neuronal vulnerability in Parkinson's spectrum (PD/PDD/DLB) and other neurodegenerative diseases.
MeSH term(s)	Animals ; Autophagy/physiology ; Humans ; Mitochondria/pathology ; Mitophagy/physiology ; Neurons/pathology ; Parkinson Disease/pathology
Language	English
Publishing date	2018-04-04
Publishing country	Ireland
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	194929-9
ISSN	1872-7972 ; 0304-3940
ISSN (online)	1872-7972
ISSN	0304-3940
DOI	10.1016/j.neulet.2018.04.004
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Zs.A 1166: Show issues

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ab Jg. 2022: Lesesaal (EG)

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Article: Excitotoxicity, calcium and mitochondria: a triad in synaptic neurodegeneration.

Verma, Manish / Lizama, Britney N / Chu, Charleen T

Translational neurodegeneration

2022 Volume 11, Issue 1, Page(s) 3

Abstract: Glutamate is the most commonly engaged neurotransmitter in the mammalian central nervous system, acting to mediate excitatory neurotransmission. However, high levels of glutamatergic input elicit excitotoxicity, contributing to neuronal cell death ... ...

Abstract	Glutamate is the most commonly engaged neurotransmitter in the mammalian central nervous system, acting to mediate excitatory neurotransmission. However, high levels of glutamatergic input elicit excitotoxicity, contributing to neuronal cell death following acute brain injuries such as stroke and trauma. While excitotoxic cell death has also been implicated in some neurodegenerative disease models, the role of acute apoptotic cell death remains controversial in the setting of chronic neurodegeneration. Nevertheless, it is clear that excitatory synaptic dysregulation contributes to neurodegeneration, as evidenced by protective effects of partial N-methyl-D-aspartate receptor antagonists. Here, we review evidence for sublethal excitatory injuries in relation to neurodegeneration associated with Parkinson's disease, Alzheimer's disease, amyotrophic lateral sclerosis and Huntington's disease. In contrast to classic excitotoxicity, emerging evidence implicates dysregulation of mitochondrial calcium handling in excitatory post-synaptic neurodegeneration. We discuss mechanisms that regulate mitochondrial calcium uptake and release, the impact of LRRK2, PINK1, Parkin, beta-amyloid and glucocerebrosidase on mitochondrial calcium transporters, and the role of autophagic mitochondrial loss in axodendritic shrinkage. Finally, we discuss strategies for normalizing the flux of calcium into and out of the mitochondrial matrix, thereby preventing mitochondrial calcium toxicity and excitotoxic dendritic loss. While the mechanisms that underlie increased uptake or decreased release of mitochondrial calcium vary in different model systems, a common set of strategies to normalize mitochondrial calcium flux can prevent excitatory mitochondrial toxicity and may be neuroprotective in multiple disease contexts.
MeSH term(s)	Animals ; Glutamic Acid/metabolism ; Mammals/metabolism ; Mitochondria/metabolism ; Neurodegenerative Diseases/metabolism ; Neurons/metabolism ; Parkinson Disease/metabolism
Chemical Substances	Glutamic Acid (3KX376GY7L)
Language	English
Publishing date	2022-01-25
Publishing country	England
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	2653701-1
ISSN	2047-9158
ISSN	2047-9158
DOI	10.1186/s40035-021-00278-7
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Article ; Online: Variable Phenotype of Congenital Corneal Opacities in Biallelic CYP1B1 Pathogenic Variants.

Franco, Elena / Gagrani, Meghal / Scanga, Hannah L / Areaux, Raymond G / Chu, Charleen T / Nischal, Ken K

Cornea

2023 Volume 43, Issue 2, Page(s) 195–200

Abstract: Purpose: The aim of this study is to describe the variable phenotype of congenital corneal opacities occurring in patients with biallelic CYP1B1 pathogenic variants.: Methods: A retrospective chart review was conducted to identify patients with ... ...

Abstract	Purpose: The aim of this study is to describe the variable phenotype of congenital corneal opacities occurring in patients with biallelic CYP1B1 pathogenic variants. Methods: A retrospective chart review was conducted to identify patients with congenital corneal opacities and CYP1B1 pathogenic variants seen at UPMC Children's Hospital of Pittsburgh. Ophthalmic examination, high-frequency ultrasound, anterior segment optical coherence tomography, histopathologic images, and details of genetic testing were reviewed. Results: Three children were identified. All presented with raised intraocular pressure. Two patients showed bilateral limbus-to-limbus avascular corneal opacification that did not resolve with intraocular pressure control; 1 showed unilateral avascular corneal opacity with a crescent of clear cornea, iridocorneal adhesions, iridolenticular adhesions, and classical features of congenital glaucoma in the fellow eye (enlarged corneal diameter, Haab striae, and clearing of the corneal clouding with appropriate intraocular pressure control). The first 2 patients were visually rehabilitated with penetrating keratoplasty. Histopathology revealed distinct features: a variably keratinized epithelium; a thick but discontinuous Bowman-like layer with areas of disruption and abnormal cellularity; Descemet membrane, when observed, showed reduced endothelial cells; and no pathological changes of Haab striae were identified. Two patients had compound heterozygous pathogenic variants in CYP1B1 causing premature stop codons, whereas 1 was homozygous for a pathogenic missense variant. Conclusions: Congenital corneal opacities seen in biallelic CYP1B1 pathogenic variants have a variable phenotype. One is that commonly termed as Peters anomaly type 1 (with iridocorneal adhesions, with or without iridolenticular adhesions) and the other is a limbus-to-limbus opacity, termed CYP1B1 cytopathy. Clinicians should be aware of this phenotypic variability.
MeSH term(s)	Child ; Humans ; Retrospective Studies ; Endothelial Cells ; Corneal Opacity/diagnosis ; Corneal Opacity/genetics ; Corneal Opacity/surgery ; Corneal Diseases/diagnosis ; Corneal Diseases/genetics ; Phenotype ; Biological Variation, Population ; Cytochrome P-450 CYP1B1/genetics
Chemical Substances	CYP1B1 protein, human (EC 1.14.14.1) ; Cytochrome P-450 CYP1B1 (EC 1.14.14.1)
Language	English
Publishing date	2023-10-03
Publishing country	United States
Document type	Journal Article
ZDB-ID	604826-2
ISSN	1536-4798 ; 0277-3740
ISSN (online)	1536-4798
ISSN	0277-3740
DOI	10.1097/ICO.0000000000003395
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Article ; Online: Congenital corneal staphyloma in 8q21.11 microdeletion syndrome.

Franco, Elena / Scanga, Hannah L / Jacob, Soosan / Chu, Charleen T / Nischal, Ken K

Ophthalmic genetics

2022 Volume 44, Issue 2, Page(s) 147–151

Abstract: Background: Although 8q21.11 microdeletion syndrome (8q21.11 DS) has been reported in association with congenital corneal opacities, reports of the clinicopathological features and management are scarce.: Methods: We reviewed medical records ... ...

Abstract	Background: Although 8q21.11 microdeletion syndrome (8q21.11 DS) has been reported in association with congenital corneal opacities, reports of the clinicopathological features and management are scarce. Methods: We reviewed medical records including ophthalmic evaluations, imaging, operative reports, and pathology reports of two unrelated patients referred to the Ophthalmology Clinic of UPMC Children's Hospital of Pittsburgh with a cytogenetic diagnosis of 8q21.11 DS. Results: Ophthalmological evaluation of both children revealed bilateral enlarged, staphylomatous, and cloudy corneas with neovascularization. These findings were consistent with the diagnosis of congenital corneal staphyloma (CCS). In one patient, anterior segment optical coherence tomography and high-frequency ultrasound revealed materials consistent with lens remnants embedded in the cornea; this was confirmed by histopathology. In the second patient, lens was found to be adherent to the cornea during surgery. One eye underwent enucleation for corneal perforation secondary to elevated intraocular pressure. In the other eyes, treatment consisted of penetrating keratoplasty combined with vitrectomy. Ahmed tube was subsequently placed to control intraocular pressure. Conclusion: 8q21.11 microdeletion syndrome can be associated with bilateral CCS, likely related to a combination of anterior segment developmental anomalies and elevated intraocular pressure. Tectonic penetrating keratoplasty is necessary to prevent corneal perforation, together with a strict control of the intraocular pressure.
MeSH term(s)	Child ; Humans ; Chromosome Disorders/pathology ; Cornea/pathology ; Corneal Opacity/diagnosis ; Corneal Perforation/complications ; Corneal Perforation/pathology ; Corneal Perforation/surgery ; Eye Abnormalities/diagnosis ; Glaucoma/pathology ; Keratoplasty, Penetrating/methods
Language	English
Publishing date	2022-11-07
Publishing country	England
Document type	Journal Article
ZDB-ID	1199279-7
ISSN	1744-5094 ; 0167-6784 ; 1381-6810
ISSN (online)	1744-5094
ISSN	0167-6784 ; 1381-6810
DOI	10.1080/13816810.2022.2127152
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Article ; Online: Transcriptome from opaque cornea of Fanconi anemia patient uncovers fibrosis and two connected players

Bharesh K. Chauhan / Anagha Medsinge / Hannah L. Scanga / Charleen T. Chu / Ken K. Nischal

Molecular Genetics and Metabolism Reports, Vol 26, Iss , Pp 100712- (2021)

2021

Abstract: Congenital corneal opacities (CCO) are a group of blinding corneal disorders, where the underlying molecular mechanisms are poorly understood. Phenotyping through specialized imaging and histopathology analysis, together with assessment of key ... ...

Abstract	Congenital corneal opacities (CCO) are a group of blinding corneal disorders, where the underlying molecular mechanisms are poorly understood. Phenotyping through specialized imaging and histopathology analysis, together with assessment of key transcriptomic changes (including glycosaminoglycan metabolic enzymes) in cornea(s) with CCO from a case of Fanconi anemia is the approach taken in this study to identify causal mechanisms. Based on our findings, we propose a novel mechanism and two key players contributing to CCO.
Keywords	Corneal opacity ; Transcriptome analysis ; Fibrosis ; Basement membrane disruption ; Glycosaminoglycans ; Medicine (General) ; R5-920 ; Biology (General) ; QH301-705.5
Language	English
Publishing date	2021-03-01T00:00:00Z
Publisher	Elsevier
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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