Dimitri Krainc
Researcher
Dimitri Krainc's AcademicInfluence.com Rankings


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Philosophy Biology
Dimitri Krainc's Degrees
- PhD Neuroscience University of Chicago
- Doctorate Medicine University of Chicago
Why Is Dimitri Krainc Influential?
(Suggest an Edit or Addition)According to Wikipedia, Dimitri Krainc is the Aaron Montgomery Ward Professor and Chairman of the Ken & Ruth Davee Department of Neurology and Director of the Simpson Querrey Center for Neurogenetics at Northwestern University Feinberg School of Medicine. He completed his research and clinical training at the Massachusetts General Hospital and Harvard Medical School, where he served on faculty until relocating to Northwestern in 2013. He was awarded the Javits Neuroscience Investigator Award and outstanding investigator award from NIH, and is President-elect of the American Neurological Association. Krainc was elected to the Association of American Physicians and the National Academy of Medicine for what NAM described as “groundbreaking discoveries in the area of neurodegenerative disorders." He is the principal founding scientist of biotech companies Lysosomal Therapeutics and Vanqua Bio and serves as Venture Partner at OrbiMed.
Dimitri Krainc's Published Works
Published Works
- Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (2012) (2913)
- Apoptosis and necrosis: two distinct events induced, respectively, by mild and intense insults with N-methyl-D-aspartate or nitric oxide/superoxide in cortical cell cultures. (1995) (2037)
- Defects in Adaptive Energy Metabolism with CNS-Linked Hyperactivity in PGC-1α Null Mice (2004) (1204)
- Gaucher Disease Glucocerebrosidase and α-Synuclein Form a Bidirectional Pathogenic Loop in Synucleinopathies (2011) (1070)
- A call for transparent reporting to optimize the predictive value of preclinical research (2012) (1036)
- Transcriptional Repression of PGC-1α by Mutant Huntingtin Leads to Mitochondrial Dysfunction and Neurodegeneration (2006) (990)
- Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1 (2021) (811)
- Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease (2002) (700)
- Human iPSC-based modeling of late-onset disease via progerin-induced aging. (2013) (609)
- Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. (2006) (571)
- α-synuclein toxicity in neurodegeneration: mechanism and therapeutic strategies (2017) (520)
- Dopamine oxidation mediates mitochondrial and lysosomal dysfunction in Parkinson’s disease (2017) (492)
- Pharmacological Rescue of Mitochondrial Deficits in iPSC-Derived Neural Cells from Patients with Familial Parkinson’s Disease (2012) (461)
- Mitochondria-lysosome contacts regulate mitochondrial fission via Rab7 GTP hydrolysis (2018) (442)
- Acetylation Targets Mutant Huntingtin to Autophagosomes for Degradation (2009) (418)
- Identification and Rescue of α-Synuclein Toxicity in Parkinson Patient–Derived Neurons (2013) (410)
- The selective macroautophagic degradation of aggregated proteins requires the PI3P-binding protein Alfy. (2010) (402)
- Genome-wide expression profiling of human blood reveals biomarkers for Huntington's disease. (2005) (394)
- Dysregulation of gene expression in the R6/2 model of polyglutamine disease: parallel changes in muscle and brain. (2002) (387)
- Mitochondrial Parkin Recruitment Is Impaired in Neurons Derived from Mutant PINK1 Induced Pluripotent Stem Cells (2011) (378)
- Functional Impairment in Miro Degradation and Mitophagy Is a Shared Feature in Familial and Sporadic Parkinson's Disease. (2016) (343)
- Sirt1 mediates neuroprotection from mutant huntingtin by activation of TORC1 and CREB transcriptional pathway (2011) (327)
- Rapamycin Reverses Cellular Phenotypes and Enhances Mutant Protein Clearance in Hutchinson-Gilford Progeria Syndrome Cells (2011) (325)
- Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets (2011) (305)
- α-Synuclein–induced lysosomal dysfunction occurs through disruptions in protein trafficking in human midbrain synucleinopathy models (2016) (257)
- Deficiency of ATP13A2 Leads to Lysosomal Dysfunction, α-Synuclein Accumulation, and Neurotoxicity (2012) (246)
- hMEF2C gene encodes skeletal muscle- and brain-specific transcription factors (1993) (242)
- MEF2C, a MADS/MEF2-family transcription factor expressed in a laminar distribution in cerebral cortex. (1993) (236)
- Phosphatase and Tensin Homolog (PTEN)-induced Putative Kinase 1 (PINK1)-dependent Ubiquitination of Endogenous Parkin Attenuates Mitophagy (2012) (206)
- Antiapoptotic role of the p38 mitogen-activated protein kinase-myocyte enhancer factor 2 transcription factor pathway during neuronal differentiation. (2000) (204)
- Activation of β-Glucocerebrosidase Reduces Pathological α-Synuclein and Restores Lysosomal Function in Parkinson's Patient Midbrain Neurons (2016) (191)
- In Vitro Analysis of Huntingtin-Mediated Transcriptional Repression Reveals Multiple Transcription Factor Targets (2005) (182)
- Synaptic, Mitochondrial, and Lysosomal Dysfunction in Parkinson’s Disease (2019) (164)
- Huntington's disease protein contributes to RNA-mediated gene silencing through association with Argonaute and P bodies (2008) (157)
- Parkin and PINK1 Patient iPSC-Derived Midbrain Dopamine Neurons Exhibit Mitochondrial Dysfunction and α-Synuclein Accumulation (2016) (152)
- Identification of TMEM230 mutations in familial Parkinson’s disease (2016) (143)
- Targeting H3K4 trimethylation in Huntington disease (2013) (136)
- Regulation and Function of Mitochondria-Lysosome Membrane Contact Sites in Cellular Homeostasis. (2019) (135)
- Zn²⁺ dyshomeostasis caused by loss of ATP13A2/PARK9 leads to lysosomal dysfunction and alpha-synuclein accumulation. (2014) (134)
- ATP13A2/PARK9 Regulates Secretion of Exosomes and α-Synuclein (2014) (130)
- Pathogenic effects of novel mutations in the P‐type ATPase ATP13A2 (PARK9) causing Kufor‐Rakeb syndrome, a form of early‐onset parkinsonism (2011) (119)
- Decreased association of the transcription factor Sp1 with genes downregulated in Huntington's disease (2006) (117)
- Atp13a2-deficient mice exhibit neuronal ceroid lipofuscinosis, limited α-synuclein accumulation and age-dependent sensorimotor deficits. (2013) (115)
- Parkin Modulates Endosomal Organization and Function of the Endo-Lysosomal Pathway (2016) (100)
- Mitochondria-lysosome contacts regulate mitochondrial Ca2+ dynamics via lysosomal TRPML1 (2020) (100)
- Peroxisome-Proliferator-Activated Receptor Gamma Coactivator 1 α Contributes to Dysmyelination in Experimental Models of Huntington's Disease (2011) (99)
- IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease. (2011) (98)
- Synergistic Activation of theN-Methyl-d-aspartate Receptor Subunit 1 Promoter by Myocyte Enhancer Factor 2C and Sp1* (1998) (97)
- LRRK2 kinase activity regulates lysosomal glucocerebrosidase in neurons derived from Parkinson’s disease patients (2019) (97)
- Distinct Region-Specific α-Synuclein Oligomers in A53T Transgenic Mice: Implications for Neurodegeneration (2010) (96)
- LIMP-2 expression is critical for β-glucocerebrosidase activity and α-synuclein clearance (2014) (94)
- LRRK2 phosphorylation of auxilin mediates synaptic defects in dopaminergic neurons from patients with Parkinson’s disease (2018) (93)
- Reduced Activity of AMP-Activated Protein Kinase Protects against Genetic Models of Motor Neuron Disease (2012) (91)
- Sodium phenylbutyrate in Huntington's disease: A dose‐finding study (2007) (84)
- Recessive mutations in VPS13D cause childhood onset movement disorders (2018) (82)
- Progranulin-mediated deficiency of cathepsin D results in FTD and NCL-like phenotypes in neurons derived from FTD patients (2017) (82)
- Erythropoietin gene regulation depends on heme-dependent oxygen sensing and assembly of interacting transcription factors. (1997) (80)
- Rapamycin activates autophagy in Hutchinson-Gilford progeria syndrome (2012) (76)
- Analysis of blood-based gene expression in idiopathic Parkinson disease (2017) (72)
- Increased Lysosomal Exocytosis Induced by Lysosomal Ca2+ Channel Agonists Protects Human Dopaminergic Neurons from α-Synuclein Toxicity (2019) (72)
- The glycolytic enzyme, GPI, is a functionally conserved modifier of dopaminergic neurodegeneration in Parkinson's models. (2014) (72)
- Genome-wide expression analysis of peripheral blood identifies candidate biomarkers for schizophrenia. (2009) (69)
- Dopamine metabolism by a monoamine oxidase mitochondrial shuttle activates the electron transport chain (2019) (69)
- Identification of novel ATP13A2 interactors and their role in α-synuclein misfolding and toxicity. (2012) (69)
- Molecular mechanisms of α-synuclein and GBA1 in Parkinson’s disease (2018) (63)
- A modulator of wild-type glucocerebrosidase improves pathogenic phenotypes in dopaminergic neuronal models of Parkinson’s disease (2019) (59)
- Mutant Huntingtin Is Secreted via a Late Endosomal/Lysosomal Unconventional Secretory Pathway (2017) (58)
- Dysregulation of mitochondria-lysosome contacts by GBA1 dysfunction in dopaminergic neuronal models of Parkinson’s disease (2021) (56)
- Glucocerebrosidase mutations in a Serbian Parkinson's disease population (2013) (53)
- Clearance of mutant proteins as a therapeutic target in neurodegenerative diseases. (2010) (52)
- Neuronal vulnerability in Parkinson disease: Should the focus be on axons and synaptic terminals? (2019) (51)
- Sirt 1 mediates neuroprotection from mutant huntingtin by activation of the TORC 1 and CREB transcriptional pathway Citation (2012) (50)
- Acid ceramidase inhibition ameliorates &agr;-synuclein accumulation upon loss of GBA1 function (2018) (48)
- Lysosomal Regulation of Inter-mitochondrial Contact Fate and Motility in Charcot-Marie-Tooth Type 2. (2019) (46)
- Lysosomal trafficking defects link Parkinson's disease with Gaucher's disease (2016) (46)
- Iron overload is accompanied by mitochondrial and lysosomal dysfunction in WDR45 mutant cells (2018) (43)
- Astrocytes Protect Human Dopaminergic Neurons from α-Synuclein Accumulation and Propagation (2020) (43)
- Characterization of the complex formed by β-glucocerebrosidase and the lysosomal integral membrane protein type-2 (2016) (41)
- The Parkinson’s disease-linked protein TMEM230 is required for Rab8a-mediated secretory vesicle trafficking and retromer trafficking (2017) (39)
- Preserving Lysosomal Function in the Aging Brain: Insights from Neurodegeneration (2019) (38)
- Progranulin mutations result in impaired processing of prosaposin and reduced glucocerebrosidase activity. (2019) (37)
- Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein (2017) (35)
- Gene expression changes in blood as a putative biomarker for Huntington's disease (2009) (35)
- Evidence of TAF1 dysfunction in peripheral models of X-linked dystonia-parkinsonism (2016) (35)
- Emerging links between pediatric lysosomal storage diseases and adult parkinsonism (2019) (35)
- Histamine N-methyltransferase Thr105Ile polymorphism is associated with Parkinson's disease (2012) (34)
- Identification of ArgBP1, an Arg protein tyrosine kinase binding protein that is the human homologue of a CNS-specific Xenopus gene. (1996) (33)
- Erratum to: Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition) (Autophagy, 12, 1, 1-222, 10.1080/15548627.2015.1100356 (2016) (33)
- Lysosomal integral membrane protein-2 as a phospholipid receptor revealed by biophysical and cellular studies (2017) (33)
- Neuronal activity and secreted amyloid β lead to altered amyloid β precursor protein and presenilin 1 interactions (2013) (32)
- Lysosomal dysfunction in neurodegeneration (2012) (32)
- Editorial :Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). DOI: 10.1080/15548627.2015.1100356;WOS:000373595400001; 2-s2.0-85013763791&;PMID: 26799652 (2016) (31)
- Transcriptional Abnormalities in Huntington's Disease (2006) (29)
- Detection of Free and Protein-Bound ortho-Quinones by Near-Infrared Fluorescence. (2016) (28)
- Gelator length precisely tunes supramolecular hydrogel stiffness and neuronal phenotype in 3D culture. (2020) (24)
- Use of a two-hybrid system to investigate molecular interactions of GAP-43. (1996) (23)
- Assessing the relationship between monoallelic PRKN mutations and Parkinson’s risk (2021) (21)
- The Convergence of Alpha-Synuclein, Mitochondrial, and Lysosomal Pathways in Vulnerability of Midbrain Dopaminergic Neurons in Parkinson’s Disease (2020) (21)
- Organoid and pluripotent stem cells in Parkinson’s disease modeling: an expert view on their value to drug discovery (2020) (20)
- Lysosomal Proteins as a Therapeutic Target in Neurodegeneration. (2017) (19)
- Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors (2010) (19)
- β-Glucocerebrosidase Modulators Promote Dimerization of β-Glucocerebrosidase and Reveal an Allosteric Binding Site. (2018) (19)
- A patient-based model of RNA mis-splicing uncovers treatment targets in Parkinson’s disease (2020) (18)
- Development of targeted therapies for Parkinson’s disease and related synucleinopathies (2014) (18)
- Micropatterning Facilitates the Long‐Term Growth and Analysis of iPSC‐Derived Individual Human Neurons and Neuronal Networks (2016) (17)
- Techniques for Distinguishing Apoptosis from Necrosis in Cerebrocortical and Cerebellar Neurons (1997) (17)
- Translational research in neurology and neuroscience 2011: movement disorders. (2011) (15)
- A PDE10A de novo mutation causes childhood‐onset chorea with diurnal fluctuations (2017) (13)
- Evaluation of Strategies for Measuring Lysosomal Glucocerebrosidase Activity (2021) (13)
- Targeted Gene Repair and Its Application to Neurodegenerative Disorders (2002) (13)
- Design and Synthesis of Potent Quinazolines as Selective β-Glucocerebrosidase Modulators. (2016) (12)
- Dominant mutations in MIEF1 affect mitochondrial dynamics and cause a singular late onset optic neuropathy (2021) (12)
- Conversion of Quinazoline Modulators from Inhibitors to Activators of β-Glucocerebrosidase. (2019) (12)
- The role of dopamine in the pathogenesis of GBA1-linked Parkinson's disease (2019) (12)
- Functional assays for the assessment of the pathogenicity of variants of GOSR2, an ER-to-Golgi SNARE involved in progressive myoclonus epilepsies (2017) (10)
- Huntington's disease: tagged for clearance (2010) (9)
- Mendelian Randomisation Study of Smoking, Alcohol, and Coffee Drinking in Relation to Parkinson's Disease. (2021) (9)
- BACE1-cleavage of Sez6 and Sez6L is elevated in Niemann-Pick type C disease mouse brains (2018) (9)
- MED27 Variants Cause Developmental Delay, Dystonia, and Cerebellar Hypoplasia (2021) (9)
- Challenges to neurology residency education in today's health care environment (2016) (9)
- Identification of ASCL1 as a determinant for human iPSC-derived dopaminergic neurons (2021) (8)
- Alzheimer gene APOE ε4 linked to brain development in infants. (2014) (7)
- Heterozygous GBA D409V and ATP13a2 mutations do not exacerbate pathological α-synuclein spread in the prodromal preformed fibrils model in young mice (2021) (7)
- Pharmacological upregulation of PGC1α in oligodendrocytes: implications for Huntington's Disease. (2013) (6)
- Long-term clinical outcomes after fetal cell transplantation in parkinson disease: implications for the future of cell therapy. (2014) (6)
- IRS 2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease (2018) (5)
- Fluctuations in cell density alter protein markers of multiple cellular compartments, confounding experimental outcomes (2019) (5)
- Direct targeting of wild-type glucocerebrosidase by antipsychotic quetiapine improves pathogenic phenotypes in Parkinson’s disease models (2021) (5)
- Methamphetamine‐induced mitochondrial oxidant stress mediated by monoamine oxidase metabolism of dopamine (2017) (5)
- Replication assessment of NUS1 variants in Parkinson's disease (2020) (5)
- Dopamine oxidation mediates a human-specific cascade of mitochondrial and lysosomal dysfunction in Parkinson's disease. (2017) (5)
- Bi-allelic variants in TSPOAP1, encoding the active zone protein RIMBP1, cause autosomal recessive dystonia (2020) (5)
- Dairy Intake and Parkinson's Disease: A Mendelian Randomization Study (2022) (4)
- Dysregulation of organelle membrane contact sites in neurological diseases (2022) (4)
- Genome-wide Association and Meta-analysis of Age at Onset in Parkinson Disease (2022) (4)
- Assignment of human myocyte-specific enhancer binding factor 2C (hMEF2C) to human chromosome 5q14 and evidence that MEF2C is evolutionarily conserved. (1995) (3)
- Glucocerebrosidase mutations: tipping point toward Parkinson disease and dementia? (2013) (3)
- Untangling alpha synuclein fibrils by graphene quantum dots (2018) (3)
- Correction to: Preserving Lysosomal Function in the Aging Brain: Insights from Neurodegeneration (2019) (3)
- Mutant Huntingtin Secretion in Neuro2A Cells and Rat Primary Cortical Neurons. (2018) (3)
- Mid51/Fis1 mitochondrial oligomerization complex drives lysosomal untethering and network dynamics (2022) (3)
- Modeling Brain Pathology of Niemann‐Pick Disease Type C Using Patient‐Derived Neurons (2021) (3)
- Author Correction: Dopamine metabolism by a monoamine oxidase mitochondrial shuttle activates the electron transport chain (2020) (2)
- Genome-wide contribution of common short-tandem repeats to Parkinson's disease genetic risk (2021) (2)
- Whole-exome analysis in Parkinson’s disease reveals a high burden of ultra rare variants in early onset cases (2020) (2)
- Inhibition of PIP 4 K ameliorates the pathological effects of mutant huntingtin protein 1 (2017) (2)
- Glucocerebrosidase dysfunction in neurodegenerative disease. (2021) (2)
- Lysosomal ceramides regulate Cathepsin B-mediated processing of saposin C and glucocerebrosidase activity. (2022) (2)
- Movement disorders in 2011: Translating new research findings into clinical practice (2012) (1)
- Mechanisms of Glucocerebrosidase Dysfunction in Parkinson's Disease. (2023) (1)
- ASSESSING THE RELATIONSHIP BETWEEN MONOALLELIC PARK2 MUTATIONS AND PARKINSONS RISK (2020) (1)
- Correction to Conversion of Quinazoline Modulators from Inhibitors to Activators of β-Glucocerebrosidase. (2019) (1)
- DEFINING CANDIDATE PARKINSONS DISEASE GENES THROUGH THE ANALYSIS OF GENOME-WIDE HOMOZYGOSITY (2020) (1)
- Genome-wide epistasis analysis in Parkinson's disease between populations with different genetic ancestry reveals significant variant-variant interactions (2022) (1)
- Apoptosis andnecrosis: Twodistinct eventsinduced, respectively, bymildandintense insults withN-methyl-D-aspartate ornitric oxide/superoxide incortical cell cultures (1995) (1)
- Human Neuron Cultures: Micropatterning Facilitates the Long‐Term Growth and Analysis of iPSC‐Derived Individual Human Neurons and Neuronal Networks (Adv. Healthcare Mater. 15/2016) (2016) (0)
- Gene-based burden analysis of damaging private variants in PRKN, PARK7 and PINK1 in Parkinson's disease cohorts of European descent (2022) (0)
- Lysosomal integral membrane protein-2 as a phospholipid receptor revealed by biophysical and cellular studies (2017) (0)
- Sp1 and Huntington’s Disease (2005) (0)
- Correction to: Preserving Lysosomal Function in the Aging Brain: Insights from Neurodegeneration (2019) (0)
- Reviewing Editors (2016) (0)
- Peripheral Sphingolipids as Potential Biomarkers of Parkinson disease Including Sex-Related Differences (P3-11.007) (2023) (0)
- Evidence of TAF1 dysfunction in peripheral models of X-linked dystonia-parkinsonism (2016) (0)
- Dominant mutations in MIEF1 affect mitochondrial dynamics and cause a singular late onset optic neuropathy (2021) (0)
- The effect of impaired biogenesis and release of exosomes on the alpha synuclein accumulation in Kufor-Rakeb syndrome (2016) (0)
- Assessment of private variants in PRKN, PARK7 and PINK1 in Parkinson's disease (2022) (0)
- Variants in ATP5F1B are associated with dominantly inherited dystonia. (2023) (0)
- Genome-wide Association and Meta-analysis of Age at Onset in Parkinson Disease (2022) (0)
- Molecular mechanisms of α-synuclein and GBA1 in Parkinson’s disease (2017) (0)
- Corrigendum: Iron overload is accompanied by mitochondrial and lysosomal dysfunction in WDR45 mutant cells (Brain (2018) 141 (3052-3064) DOI: 10.1093/brain/awy230) (2019) (0)
- Octopamine metabolically reprograms astrocytes to confer neuroprotection against α-synuclein (2022) (0)
- Title : Challenges to Neurology Residency Education in Today ’ s Healthcare Environment Running Title : Challenges to Residency Education (2016) (0)
- Kan Cao Clearance in Hutchinson-Gilford Progeria Syndrome Cells Rapamycin Reverses Cellular Phenotypes and Enhances Mutant Protein (2011) (0)
- A Mechanistic Connection Between Gaucher Disease and the Synucleinopathies (2012) (0)
- ReducedActivityofAMP-ActivatedProteinKinaseProtects againstGeneticModelsofMotorNeuronDisease (2012) (0)
- ASSESSING THE RELATIONSHIP BETWEEN MONOALLELIC PRKN MUTATIONS AND PARKINSON’S RISK AUTHORSHIP (2020) (0)
- Expanding the spectrum of KCNJ6‐related disorders: Milder phenotype with pathological startle responses (2022) (0)
- Preserving Lysosomal Function in the Aging Brain: Insights from Neurodegeneration (2019) (0)
- Author Correction: Dopamine metabolism by a monoamine oxidase mitochondrial shuttle activates the electron transport chain (2020) (0)
- Extracellular transportation of α-synuclein by HLA class II molecules. (2022) (0)
- Crystal structure of glucocerebrosidase with an inhibitory quinazoline modulator (2017) (0)
- Novel THAP1 missense variant with incomplete penetrance in a case of generalized young onset dystonia showing good response to deep brain stimulation. (2022) (0)
- Yeun Chung Derived Neurons − Patient-Synuclein Toxicity in Parkinson α Identification and Rescue (2016) (0)
- Dopamine metabolism by a monoamine oxidase mitochondrial shuttle activates the electron transport chain (2019) (0)
- Novel Approach to Tracking Mutant Huntingtin in Biosamples. (2018) (0)
- Chung Derived Neurons − Patient-Synuclein Toxicity in Parkinson α Identification and Rescue of (2014) (0)
- High priority publications on Parkinson's disease in 2017 (2018) (0)
- Misregulation of mitochondria–lysosome contact sites in glucocerebrosidase (GBA) Parkinson's patient neurons (2019) (0)
- Novel bi-allelic FBXO7 variants in a family with early-onset typical Parkinson's disease. (2022) (0)
- Investigation of Shared Genetic Risk Factors Between Parkinson's Disease and Cancers (2023) (0)
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