Isabel Faust
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View article: XYLT1 Deficiency of Human Mesenchymal Stem Cells: Impact on Osteogenic, Chondrogenic, and Adipogenic Differentiation
XYLT1 Deficiency of Human Mesenchymal Stem Cells: Impact on Osteogenic, Chondrogenic, and Adipogenic Differentiation Open
Xylosyltransferase-I (XT-I) plays a crucial role in skeletal development and cartilage integrity. An XT-I deficiency is linked to severe bone disorders, such as Desbuquois dysplasia type 2. While animal models have provided insights into X…
View article: Xylosyltransferase-Deficiency in Human Dermal Fibroblasts Induces Compensatory Myofibroblast Differentiation and Long-Term ECM Reduction
Xylosyltransferase-Deficiency in Human Dermal Fibroblasts Induces Compensatory Myofibroblast Differentiation and Long-Term ECM Reduction Open
Desbuquois dysplasia type 2 (DBQD2) and spondylo-ocular syndrome (SOS) are autosomal recessive disorders affecting the extracellular matrix (ECM) and categorized as glycosaminoglycan (GAG) linkeropathies. Linkeropathies result from mutatio…
View article: A novel SPE-UPLC-MS/MS-based assay for the selective, simultaneous quantification of xylosyltransferase-I and -II activity
A novel SPE-UPLC-MS/MS-based assay for the selective, simultaneous quantification of xylosyltransferase-I and -II activity Open
Xylosyltransferase-I and -II (XT-I, -II) possess a central role during the glycosylation of proteoglycans (PGs). They catalyze the formation of an O-glycosidic bond between the xylosyl residue of uridinediphosphate-xylose and the core prot…
View article: Understanding of arthrofibrosis: New explorative insights into extracellular matrix remodeling of synovial fibroblasts
Understanding of arthrofibrosis: New explorative insights into extracellular matrix remodeling of synovial fibroblasts Open
Arthrofibrosis following total knee arthroplasty is a fibroproliferative joint disorder marked by dysregulated biosynthesis of extracellular matrix proteins, such as collagens and proteoglycans. The underlying cellular events remain incomp…
View article: The Consideration of Pseudoxanthoma Elasticum as a Progeria Syndrome
The Consideration of Pseudoxanthoma Elasticum as a Progeria Syndrome Open
Background: Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disorder caused by mutations in the ATP-binding cassette sub-family C member 6 (ABCC6) gene. Patients with PXE show molecular and clinical characteristics of known pr…
View article: Human Xylosyltransferase I—An Important Linker between Acute Senescence and Fibrogenesis
Human Xylosyltransferase I—An Important Linker between Acute Senescence and Fibrogenesis Open
The human xylosyltransferase isoform XT-I catalyzes the initial step in proteoglycan biosynthesis and represents a biomarker of myofibroblast differentiation. Furthermore, XT-I overexpression is associated with fibrosis, whereby a fibrotic…
View article: The Human Myofibroblast Marker Xylosyltransferase-I: A New Indicator for Macrophage Polarization
The Human Myofibroblast Marker Xylosyltransferase-I: A New Indicator for Macrophage Polarization Open
Chronic inflammation and excessive synthesis of extracellular matrix components, such as proteoglycans (PG), by fibroblast- or macrophage-derived myofibroblasts are the hallmarks of fibrotic diseases, including systemic sclerosis (SSc). Hu…
View article: The Impact of Inflammatory Stimuli on Xylosyltransferase-I Regulation in Primary Human Dermal Fibroblasts
The Impact of Inflammatory Stimuli on Xylosyltransferase-I Regulation in Primary Human Dermal Fibroblasts Open
Inflammation plays a vital role in regulating fibrotic processes. Beside their classical role in extracellular matrix synthesis and remodeling, fibroblasts act as immune sentinel cells participating in regulating immune responses. The huma…
View article: First Characterization of Human Dermal Fibroblasts Showing a Decreased Xylosyltransferase-I Expression Induced by the CRISPR/Cas9 System
First Characterization of Human Dermal Fibroblasts Showing a Decreased Xylosyltransferase-I Expression Induced by the CRISPR/Cas9 System Open
Background: Xylosyltransferases-I and II (XT-I and XT-II) catalyze the initial and rate limiting step of the proteoglycan (PG) biosynthesis and therefore have an import impact on the homeostasis of the extracellular matrix (ECM). The reaso…
View article: Statins as a Therapeutic Approach for the Treatment of Pseudoxanthoma Elasticum Patients: Evaluation of the Spectrum Efficacy of Atorvastatin In Vitro
Statins as a Therapeutic Approach for the Treatment of Pseudoxanthoma Elasticum Patients: Evaluation of the Spectrum Efficacy of Atorvastatin In Vitro Open
Pseudoxanthoma elasticum (PXE) is an autosomal recessive disorder caused by mutations in the ATP-binding cassette sub-family C member 6 gene. Our previous studies revealed that PXE might be associated with premature aging. Treatment with s…
View article: Linking ABCC6 Deficiency in Primary Human Dermal Fibroblasts of PXE Patients to p21-Mediated Premature Cellular Senescence and the Development of a Proinflammatory Secretory Phenotype
Linking ABCC6 Deficiency in Primary Human Dermal Fibroblasts of PXE Patients to p21-Mediated Premature Cellular Senescence and the Development of a Proinflammatory Secretory Phenotype Open
Pseudoxanthoma elasticum (PXE) is a rare autosomal-recessive disorder that is mainly caused by mutations in the ATP-binding cassette sub-family C member 6 (ABCC6) gene. Clinically PXE is characterized by a loss of skin elasticity, arterios…
View article: Identification of Putative Non-Substrate-Based XT-I Inhibitors by Natural Product Library Screening
Identification of Putative Non-Substrate-Based XT-I Inhibitors by Natural Product Library Screening Open
Fibroproliferative diseases are characterized by excessive accumulation of extracellular matrix (ECM) components leading to organ dysfunction. This process is characterized by an increase in myofibroblast content and enzyme activity of xyl…
View article: Blood Serum Stimulates p38-Mediated Proliferation and Changes in Global Gene Expression of Adult Human Cardiac Stem Cells
Blood Serum Stimulates p38-Mediated Proliferation and Changes in Global Gene Expression of Adult Human Cardiac Stem Cells Open
During aging, senescent cells accumulate in various tissues accompanied by decreased regenerative capacities of quiescent stem cells, resulting in deteriorated organ function and overall degeneration. In this regard, the adult human heart …
View article: Activin A-Mediated Regulation of XT-I in Human Skin Fibroblasts
Activin A-Mediated Regulation of XT-I in Human Skin Fibroblasts Open
Fibrosis is a fundamental feature of systemic sclerosis (SSc) and is characterized by excessive accumulation of extracellular matrix components like proteoglycans (PG) or collagens in skin and internal organs. Serum analysis from SSc patie…
View article: Cellular and Molecular Biomarkers Indicate Premature Aging in Pseudoxanthoma Elasticum Patients
Cellular and Molecular Biomarkers Indicate Premature Aging in Pseudoxanthoma Elasticum Patients Open
The molecular processes of aging are very heterogenic and not fully understood. Studies on rare progeria syndromes, which display an accelerated progression of physiological aging, can help to get a better understanding. Pseudoxanthoma ela…
View article: Acute Retinopathy in Pseudoxanthoma Elasticum
Acute Retinopathy in Pseudoxanthoma Elasticum Open
Acute retinopathy in patients with PXE may occur, with symptoms ranging from short-term, reversible alterations to irreversible vision loss; these findings contribute to understanding the variable ocular disease progression in PXE and prov…
View article: Identification of a human adult cardiac stem cell population with neural crest origin
Identification of a human adult cardiac stem cell population with neural crest origin Open
Cardiovascular diseases are the major cause of death worldwide, emphasizing the necessity to better understand adult human cardiac cell biology and development. Although the adult heart was considered as a terminally differentiated organ, …
View article: Abcc6 deficiency in mice leads to altered ABC transporter gene expression in metabolic active tissues
Abcc6 deficiency in mice leads to altered ABC transporter gene expression in metabolic active tissues Open
These data support previous findings that Abcc6 deficiency leads to an altered gene expression of other ABC transporters depending on the status of disease progression. The increased expression of fatty acid, bile salt and cholesterol/ster…
View article: Pathobiochemistry of arthrofibrotic remodeling
Pathobiochemistry of arthrofibrotic remodeling Open
Aims and Objectives: Arthrofibrosis is defined as painful impairment of joint flexibility due to fibrotic tissue remodeling after joint trauma or surgery. The incidence of arthrofibrosis after knee replacement surgery is 5 to 10%. Although…
View article: Arthrofibrotic remodeling after knee replacement surgery, a stress-related disease? - Clinic, biochemical model, therapy, research -
Arthrofibrotic remodeling after knee replacement surgery, a stress-related disease? - Clinic, biochemical model, therapy, research - Open
Aims and Objectives: The predicted incidence of arthrofibrosis after knee replacement surgery is 5 - 10%. Arthrofibrosis is defined as painful impairment of joint flexibility occurring postoperatively. To counteract symptoms and to accompl…