retinitis pigmentosa/protease

Länken sparas på Urklipp

Välj sorteringstyp

Sida 1 från 27 resultat

Differential Contribution of Calcium-Activated Proteases and ER-Stress in Three Mouse Models of Retinitis Pigmentosa Expressing P23H Mutant RHO.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Autosomal dominant retinitis pigmentosa (adRP) is mainly caused by mutations responsible for rhodopsin (RHO) misfolding. Although it was previously proved that unfolded RHO is retained into the endoplasmatic reticulum (ER) eliciting ER-stress, consequent mechanisms underlying photoreceptor

Dynamics of the rhomboid-like protein RHBDD2 expression in mouse retina and involvement of its human ortholog in retinitis pigmentosa.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

The novel rhomboid-like protein RHBDD2 is distantly related to rhomboid proteins, a group of highly specialized membrane-bound proteases that catalyze regulated intramembrane proteolysis. In retina, RHBDD2 is expressed from embryonic stages to adulthood, and its levels show age-dependent changes.

rd1 Mouse retina shows an imbalance in the activity of cysteine protease cathepsins and their endogenous inhibitor cystatin C.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

OBJECTIVE To compare in vivo levels, spatial localization, and in vitro secretion of cysteine protease cathepsins and cystatin C (cysC) in the retinal degeneration 1 (rd1) mouse model of retinitis pigmentosa and control (wt) mouse retinas. METHODS The spatial localization, protein contents, cysC

TOPORS, a Dual E3 Ubiquitin and Sumo1 Ligase, Interacts with 26 S Protease Regulatory Subunit 4, Encoded by the PSMC1 Gene.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

The significance of the ubiquitin-proteasome system (UPS) for protein degradation has been highlighted in the context of neurodegenerative diseases, including retinal dystrophies. TOPORS, a dual E3 ubiquitin and SUMO1 ligase, forms a component of the UPS and selected substrates for its enzymatic

Intravitreal injection or topical eye-drop application of a μ-calpain C2L domain peptide protects against photoreceptor cell death in Royal College of Surgeons' rats, a model of retinitis pigmentosa.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Mitochondrial μ-calpain initiates apoptosis-inducing factor (AIF)-dependent apoptosis in retinal photoreceptor degeneration. Mitochondrial μ-calpain inhibitors may represent therapeutic targets for the disease. Therefore, we sought to identify inhibitors of mitochondrial calpains and determine their

Loss of HCN1 enhances disease progression in mouse models of CNG channel-linked retinitis pigmentosa and achromatopsia.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Most inherited blinding diseases are characterized by compromised retinal function and progressive degeneration of photoreceptors. However, the factors that affect the life span of photoreceptors in such degenerative retinal diseases are rather poorly understood. Here, we explore the role of

Lysosomal membrane permeabilization and autophagy blockade contribute to photoreceptor cell death in a mouse model of retinitis pigmentosa.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Retinitis pigmentosa is a group of hereditary retinal dystrophies that normally result in photoreceptor cell death and vision loss both in animal models and in affected patients. The rd10 mouse, which carries a missense mutation in the Pde6b gene, has been used to characterize the underlying

A homozygous missense mutation in the IRBP gene (RBP3) associated with autosomal recessive retinitis pigmentosa.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

OBJECTIVE Interphotoreceptor retinoid-binding protein (IRBP) has been considered essential for normal rod and cone function, as it mediates the transport of retinoids between the photoreceptors and the retinal pigment epithelium. This study was performed to determine whether mutations in the IRBP

Activated mTORC1 promotes long-term cone survival in retinitis pigmentosa mice.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Retinitis pigmentosa (RP) is an inherited photoreceptor degenerative disorder that results in blindness. The disease is often caused by mutations in genes that are specific to rod photoreceptors; however, blindness results from the secondary loss of cones by a still unknown mechanism. Here, we

Loss of the cone-enriched caspase-7 does not affect secondary cone death in retinitis pigmentosa.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

The apoptotic mechanisms responsible for secondary cone death in retinitis pigmentosa (RP) remain largely unknown. The cone-enriched apoptotic protease caspase-7 (Casp7) is thought to be triggered by endoplasmic reticulum (ER) stress and plays a pivotal role in mice deficient in the cone cyclic

Low glutathione peroxidase in rd1 mouse retina increases oxidative stress and proteases.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Malondialdehyde, reduced glutathione, glutathione peroxidase, glutathione reductase and cysteine protease cathepsins at postnatal (PN) days 2, 7, 14, 21 and 28 in controls (wt) and the retinal degeneration 1 (rd1) mouse model for retinitis pigmentosa retinas were measured to determine oxidative

Cut-like homeobox 1 (CUX1) regulates expression of the fat mass and obesity-associated and retinitis pigmentosa GTPase regulator-interacting protein-1-like (RPGRIP1L) genes and coordinates leptin receptor signaling.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

The first intron of FTO contains common single nucleotide polymorphisms associated with body weight and adiposity in humans. In an effort to identify the molecular basis for this association, we discovered that FTO and RPGRIP1L (a ciliary gene located in close proximity to the transcriptional start

Pigment epithelium-derived factor delays the death of photoreceptors in mouse models of inherited retinal degenerations.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Pigment epithelium-derived factor (PEDF) is a member of the serine protease inhibitor superfamily produced by retinal pigment epithelial cells in the developing and adult retina. In vitro, it induces neuronal differentiation of retinoblastoma cells and promotes survival of cerebellar granule

Activation of multiple pathways during photoreceptor apoptosis in the rd mouse.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

OBJECTIVE The primary purpose of this study was to characterize photoreceptor apoptosis in the rd mouse. Given that apoptosis is the final common pathway in many cases of retinal degeneration, the ability to retard or even arrest this process may ameliorate retinal disorders such as retinitis

Protein Carbonylation-Dependent Photoreceptor Cell Death Induced by N-Methyl-N-nitrosourea in Mice.

Endast registrerade användare kan översätta artiklar

Logga in Bli medlem

Retinal degenerative diseases, such as retinitis pigmentosa, are characterized by night blindness and peripheral vision loss caused by the slowly progressive loss of photoreceptor cells. A comprehensive molecular mechanism of the photoreceptor cell death remains unclear. We previously reported that

Tidigare
1
2
Nästa

Gå med på vår
facebook-sida

Herbal & Natural Medicine

Den mest kompletta databasen med medicinska örter som stöds av vetenskapen

Fungerar på 55 språk
Växtbaserade botemedel som stöds av vetenskap
Örter igenkänning av bild
Interaktiv GPS-karta - märka örter på plats (kommer snart)
Läs vetenskapliga publikationer relaterade till din sökning
Sök efter medicinska örter efter deras effekter
Organisera dina intressen och håll dig uppdaterad med nyheterna, kliniska prövningar och patent

Skriv ett symptom eller en sjukdom och läs om örter som kan hjälpa, skriv en ört och se sjukdomar och symtom den används mot.
* All information baseras på publicerad vetenskaplig forskning