pepstatin/atrofia

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Failure to prevent degeneration of insect muscles with pepstatin.

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Delayed degeneration of dystrophic and normal muscle cell cultures treated with pepstatin, leupeptin, and antipain.

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Light induced retinal degeneration activates a caspase-independent pathway involving cathepsin D.

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Neuroprotection strategies in the retina aim at interference with regulatory mechanisms of cell death. To successfully target these mechanisms it is necessary to understand the molecular pathways activated in the degenerating retina. Induced retinal degeneration models, like the light damage model,

Muscular dystrophy: inhibition of degeneration in vivo with protease inhibitors.

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The protease inhibitors leupeptin and pepstatin were used in vivo in genetically dystrophic chickens to determine their effects on the histological and biochemical changes observed in this disease. These compounds appear to delay the degeneration of muscle tissue which is characteristic of this

The neuronal ceroid-lipofuscinoses (Batten disease): a new class of lysosomal storage diseases.

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The neuronal ceroid-lipofuscinoses (Batten disease) are a group of severe neurodegenerative disorders characterized clinically by visual loss, seizures and psychomotor degeneration, and pathologically by loss of neurons and lysosomal accumulation of autofluorescent storage material resembling ageing

Oxidative stress-induced phosphorylation, degradation and aggregation of alpha-synuclein are linked to upregulated CK2 and cathepsin D.

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Intracellular accumulation of alpha-synuclein (alpha-Syn) as filamentous aggregates is a pathological feature shared by Parkinson's disease, dementia with Lewy bodies and multiple system atrophy, referred to as synucleinopathies. To understand the mechanisms underlying alpha-Syn aggregation, we

Beneficial effects of cathepsin inhibition to prevent chemotherapy-induced intestinal mucositis.

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One of the main secondary toxic side effects of anti-mitotic agents used to treat cancer patients is intestinal mucositis. Previous data showed that cathepsin D activity, contributing to the proteolytic lysosomal pathway, is up-regulated during intestinal mucositis in rats. At the same time,

Increase in cathepsin D activity in rat brain in aging.

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Cathepsin D-like activity in homogenates of five brain areas of 3-month-old and 24-month-old Fischer 344 rats was measured. With hemoglobin as substrate at pH 3.2, more than 90% of the activity was inhibited by pepstatin. In each area studied, activity was more than twice as high in the old rat

M-opsin protein degradation is inhibited by MG-132 in Rpe65⁻/⁻ retinal explant culture.

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OBJECTIVE The 65 kDa retinal pigment epithelium-specific protein, RPE65, is an essential enzyme for 11-cis-retinal synthesis in the eye. Mutations of the RPE65 gene in humans result in severe vision loss, and Rpe65(-/-) mice show early cone photoreceptor degeneration. We used an explant culture

Cathepsin D displays in vitro beta-secretase-like specificity.

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The formation of A beta and A beta-containing fragments is likely a key event in the process of neural degeneration in Alzheimer's disease. The N-terminal residue (Asp-1) of A beta and its C-terminally extended sequences is liberated from the beta-amyloid precursor protein (beta APP) by

Peripheral nerve autografts increase soleus muscle hydrolase activity.

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A peripheral nerve autograft placed on the surface of a normally innervated muscle has been shown to induce acetylcholine hypersensitivity and myofibrillar degeneration. Using a similar preparation, we determined the acid protease, alkaline protease, and N-acetylglucosaminidase activity in rat

Ca2+-dependent proteolytic activity in crab claw muscle. Effects of inhibitors and specificity for myofibrillar proteins.

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The claw closer muscle of the Bermuda land crab, Gecarcinus lateralis, undergoes a sequential atrophy and restoration during each molting cycle. We describe here the role of Ca2+-dependent proteinases in the turnover of myofibrillar protein in normal anecdysial (intermolt) claw muscle. Crab

[Neuronal ceroid lipofuscinosis. Closing chapter of a long story].

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Neuronal ceroid lipofuscinoses represent a group of diseases which has until quite recently resisted definite elucidation of the underlying defect(s) on the molecular level. The common feature of all the NCLs is a serious and progressive neurological disorder, accompanied, with only few exceptions,

Differential expression of two cathepsin Es during metamorphosis-associated remodeling of the larval to adult type epithelium in Xenopus stomach.

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Cathepsin E (CE) was purified from the foregut of Xenopus laevis tadpoles as a mature dimeric form. The purified enzyme was a typical CE among aspartic proteinases with respect to pH dependence of proteolytic activity, susceptibility to pepstatin, and having N-linked high-mannose type

Characterization of fluxional hydrogen-bonded complexes of acetic acid and acetate by NMR: geometries and isotope and solvent effects.

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1H, (2)H, and (13)C NMR spectra of enriched CH(3)(13)COOH acid without and in the presence of tetra-n-butylammonium acetate have been measured around 110 K using a liquefied Freon mixture CDF(3)/CDF(2)Cl as a solvent, as a function of the deuterium fraction in the mobile proton sites. For

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