Search Results - harry+dietz

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Treatment of fibrosis-associated pathologies through downstream TGF-β pathway inhibition

Value PropositionInhibits downstream signaling pathway of TGF-β signaling, reducing pleiotropic and off-target effects.Selectively targets tissue specific calpains, increasing efficacy as compared to current TGF-β signaling pathway inhibitory methods.Applicable to several disease models with specific focus on epithelial-to-mesenchymal transition pathologies....

Published: 3/13/2025 | Inventor(s): David Kim, Harry Dietz

Keywords(s): Biomarker, Cancers, Clinical Diagnostics, Disease Indication, Fibrosis Disorders, In Vitro Diagnostics, Mechanism-of-action Biomarker, Therapeutic Matter, Therapeutics

Category(s): Clinical and Disease Specializations, Clinical and Disease Specializations > Fibrosis, Clinical and Disease Specializations > Oncology, Technology Classifications > Diagnostics > Biomarkers, Technology Classifications > Therapeutic Modalities > Targets, Technology Classifications > Diagnostics > In Vitro Diagnostics, Technology Classifications > Diagnostics, Technology Classifications > Therapeutic Modalities

Col3a1G209S heterozygous mice

Unmet Need:Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic connective tissue disorder. Patients with vEDS present with thin, translucent skin, easy bruising, and a characteristic facial appearance. Most critically, they are at risk for spontaneous rupture of the major arteries, hollow organs, and gravid uterus. Preclinical investigation of...

Published: 3/14/2025 | Inventor(s): Harry Dietz, Caitlin Bowen, Juan Calderon Giadrosic, Mark Helmers

Keywords(s):

Category(s): Clinical and Disease Specializations > Genetic Diseases, Technology Classifications > Research Tools, Technology Classifications > Research Tools > Animal Models

Col3a1G938D heterozygous mice

Published: 3/14/2025 | Inventor(s): Harry Dietz, Caitlin Bowen

Keywords(s):

Category(s): Clinical and Disease Specializations > Genetic Diseases, Technology Classifications, Technology Classifications > Research Tools, Technology Classifications > Research Tools > Animal Models

Loeys-Dietz Syndrome (LDS) Mouse Strains (Tgfbr2G357W/+ and Tgfbr1M318R/+)

Unmet Need:Loeys-Dietz Syndrome (LDS) is a rare genetic connective tissue disorder that is characterized by a high risk for aneurysm and dissection throughout the arterial tree and phenotypically resembles Marfan Syndrome. Marfan Syndrome and related disorders, such as LDS, have an incidence of 1 in 3000 to 5000 individuals (see UpToDate). LDS is specifically...

Published: 3/14/2025 | Inventor(s): Harry Dietz, Elena MacFarlane, David Loch

Keywords(s):

Category(s): Clinical and Disease Specializations > Genetic Diseases, Technology Classifications > Research Tools, Technology Classifications > Research Tools > Animal Models

Targeted Epigenetic Therapy against Distal Regulatory Element of TGFB2 Expression for Scleroderma and Fibrotic Disease

Unmet NeedScleroderma disorders comprise a heterogeneous group of conditions linked by the presence of thickened, sclerotic skin lesions. These conditions result in a poor quality of life and have a poor prognostic outcome. In particular, systemic sclerosis (SSc) has a 10 year mortality rate of approximately 30%. However, the pathogenic mechanisms of...

Published: 3/13/2025 | Inventor(s): Harry Dietz, Joseph Shin

Keywords(s): Biomarker, Clinical Diagnostics, Diagnostic Biomarker, Disease Indication, Fibrosis Disorders, In Vitro Diagnostics, Prognostic Biomarker, Therapeutics

Category(s): Clinical and Disease Specializations, Clinical and Disease Specializations > Fibrosis, Technology Classifications > Diagnostics > Biomarkers, Technology Classifications > Diagnostics > Diagnostic Biomarkers, Technology Classifications > Diagnostics > In Vitro Diagnostics, Technology Classifications > Diagnostics > Prognostics Biomarkers, Technology Classifications > Therapeutic Modalities, Technology Classifications > Diagnostics

Conditional mouse model of pseudoxanthoma elasticum (PXE); floxed Abcc6 allele

TITLE: Conditional mouse model of pseudoxanthoma elasticum (PXE); floxed Abcc6 alleleCASE NUMBER: C13613 ABSTRACTUsing ES-cells, JHU researchers generated a PXE conditional mouse model (floxed Abcc6 allele). When bred to a CMV-cre mouse, this mouse develops robust calcification in the vibrissae (snout of the mouse). They use this mouse model to test...

Published: 3/13/2025 | Inventor(s): Harry Dietz, Shira Ziegler

Keywords(s): Basic Research Biomarker, Cardiovascular Diseases, Cell Lines, Discovery/Research Tools, Disease Indication, In Vitro Research Tool, In Vivo Research Tool, Knock In Mouse Model, Mouse Cell Lines, Mouse Model

Category(s): Technology Classifications > Research Tools > Animal Models, Technology Classifications > Research Tools > Cell Lines, Technology Classifications > Research Tools, Clinical and Disease Specializations > Cardiovascular

Fibrillin 1 Mutant Mice (W1572C Mutant Strain) and (D1545E Mutant Strain)

TITLE: Fibrillin 1 Mutant Mice (W1572C Mutant strain) and (D1545E Mutant Strain)CASE NUMBER: C13499ABSTRACTFibrillin1 D1545E Mutant StrainThese mice carry a D1545E mutation in the mouse Fbn1 gene and serve as a model of human stiff skin syndrome with increased collagen deposition in the dermis, decreased subcutaneous fat, and circulating anti-nuclear...

Published: 3/13/2025 | Inventor(s): Harry Dietz, Elizabeth Gerber

Keywords(s): Basic Research Biomarker, Cell Lines, Discovery/Research Tools, Disease Indication, In Vitro Research Tool, In Vivo Research Tool, Knock In Mouse Model, Mouse Cell Lines, Mouse Model, Skin Disorders, Translational Research Biomarker

A Ketogenic Diet Rescues Neurogenesis Defects in a Mouse Model of Kabuki Syndrome

TITLE: A Ketogenic Diet Rescues Neurogenesis Defects in a Mouse Model of Kabuki SyndromeCASE NUMBER: C13532UNMET NEEDKabuki syndrome (KS) is a rare genetic disease resulting in intellectual disability, seen in association with postnatal growth retardation, immunological dysfunction and characteristic facial features. Currently, there are...

Published: 3/13/2025 | Inventor(s): Hans Bjornsson, Joel Benjamin, Harry Dietz

Keywords(s): Discovery/Research Tools, In Vivo Research Tool, Mouse Model

Category(s): Technology Classifications > Research Tools > Animal Models, Technology Classifications > Research Tools

Histone Reporter Alleles: Tools for in vitro and in vivo Epigenetic Therapeutic Development - Treatment Method

METHODS FOR TREATING MENDELIAN DISORDERS OF THE EPIGENETICMACHINERYJHU REF: C13083Invention novelty: A genetically encoded histone reporter allele system which can be utilized for high throughput screening in in vitro cellular systems.Value Proposition: Epigenetic modifications are thought to be one of the critical mechanisms that mediate gene-environment...

Published: 3/13/2025 | Inventor(s): Harry Dietz, Hans Bjornsson

Keywords(s): Basic Research Biomarker, Biomarker, Clinical Diagnostics, Discovery/Research Tools, Disease Indication, Epigenetic, Genetic Disorders, In Vitro Diagnostics (Old), In Vitro Research Tool, In Vivo Research Tool, Mouse Model, Mouse Model Xenograft, Target-based Screening Tool

Category(s): Technology Classifications > Research Tools > Animal Models, Technology Classifications > Research Tools, Technology Classifications > Research Tools > Assays, Clinical and Disease Specializations > Genetic Diseases, Technology Classifications > Diagnostics > Biomarkers

Enabling technology for epigenetic drug development

Invention novelty: This invention describes an improved histone reporter system that enables epigenetic therapeutic development in cell-based assays and potential therapeutic monitoring in animal models.Value PropositionIt is now widely appreciated that most human diseases are contributed by interplay of genetic and epigenetic factors. Understanding...

Published: 3/13/2025 | Inventor(s): Harry Dietz, Hans Bjornsson

Keywords(s): Basic Research Biomarker, Biomarker, Clinical Diagnostics, Cloning Reagents, Discovery/Research Tools, Epigenetic, In Vitro Diagnostics, In Vitro Diagnostics (Old), In Vitro Research Tool, Nucleotide, Research Reagent, Target-based Screening Tool

Category(s): Technology Classifications > Research Tools > Nucleic Acids, Technology Classifications > Research Tools, Technology Classifications > Research Tools > Assays, Technology Classifications > Diagnostics > Biomarkers

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