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polyneuropathies/protease

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Studies of protease and protease inhibitors in familial amyloidotic polyneuropathy.

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Serum levels of 6 protease inhibitors, alpha 1-antitrypsin, Cl inactivator, alpha 2-macroglobulin, antithrombin-3, alpha 1-antichymotrypsin and inter-alpha-trypsin inhibitor were measured in patients with familial amyloidotic polyneuropathy (FAP) and a control group without neurologic disease. No

In vitro degradation of amyloid material by four proteases in tissue of a patient with familial amyloidotic polyneuropathy.

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The effects of 4 proteolytic enzymes, alpha-chymotrypsin, bromeline, collagenase, and lysozyme on amyloid tissue sections from a patient with familial amyloidotic polyneuropathy (FAP) were evaluated. Degradation of amyloid fibrils was significant with alpha-chymotrypsin, moderate with bromeline and

Diagnostic value of serum peptidome analyses for protease activated pathological conditions beyond cancer diagnosis.

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Human serum contains thousands of proteolytically derived low-molecular-weight peptide fragments (serum peptidome). The concept of utilizing the serum peptidome for cancer diagnosis has been developed. A pathological serum peptidome appears when the homeostatic balance between proteases and protease

Sensory neuropathy and metabolic risk factors in human immune deficiency virus infected South Africans receiving protease inhibitors.

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BACKGROUND Protease inhibitors (PI)s have been associated with distal sensory polyneuropathy (DSP) and metabolic complications in high-income countries. No data exist in Africans where second-line antiretroviral therapy (ART) often include PIs. METHODS We performed a cross-sectional study to assess

DJ-1 degrades transthyretin and an inactive form of DJ-1 is secreted in familial amyloidotic polyneuropathy.

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DJ-1 plays roles in transcriptional regulation and anti-oxidative stress, and loss of its function is thought to result in the onset of Parkinson's disease. DJ-1 has a protease-like structure and transthyretin (TTR), a protein causing familial amyloidotic polyneuropathy (FAP), was identified as a

Human immunodeficiency virus protease inhibitors and risk for peripheral neuropathy.

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OBJECTIVE Two recent analyses found that exposure to protease inhibitors (PIs) in the context of antiretroviral (ARV) therapy increased the risk for distal sensory polyneuropathy (DSPN) in subjects with human immunodeficiency virus (HIV) infection. These findings were supported by an in vitro model

The first case of protease-sensitive prionopathy (PSPr) in The Netherlands: a patient with an unusual GSS-like clinical phenotype.

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An atypical case of prion disease is described in a 54-year-old Dutch man, homozygous for valine at codon 129 of the prion protein gene (PRNP). The clinical phenotype was characterised by progressive dementia, spastic paraplegia and sensorimotor polyneuropathy. The disease duration was 20 months.

The protease inhibitor, alpha 1-antichymotrypsin, is a component of the brain amyloid deposits in normal aging and Alzheimer's disease.

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The purpose of this study was to characterize the nature and the origin of the Alzheimer's disease amyloid deposits. We used an amyloid antiserum to screen a human liver expression library. A positive clone was sequenced and found to code for the serine protease inhibitor alpha 1-antichymotrypsin,

IgM paraproteins with immunological specificity for a Schwann cell component and peripheral nerve myelin in patients with polyneuropathy.

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The sera of 9 patients with benign IgM paraproteinaemia and chronic sensorimotor neuropathy were tested for reactivity to human peripheral nerves by the indirect immunoperoxidase method. They reacted in very high titre (10(-3)-10(-6) with a cytoplasmic Schwann cell component, and to lesser degree,

Hypertriglyceridemia in combination antiretroviral-treated HIV-positive individuals: potential impact on HIV sensory polyneuropathy.

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OBJECTIVE in HIV populations that are aging due to improved longevity with combination antiretroviral therapy (CART), both hypertriglyceridemia (hTRG) and sensory neuropathy have become increasingly common. Sensory neuropathy is associated with substantial long-term disability and frequently

Clinical range and MRI in Creutzfeldt-Jakob disease with heterozygosity at codon 129 and prion protein type 2.

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A 68 year old woman with sporadic Creutzfeldt-Jakob disease is described, who neither showed characteristic EEG abnormalities nor a positive test of the neuronal protein 14-3-3 or neuron specific enolase (NSE) in CSF, despite a clinical presentation with ataxia of cerebellar type, rapidly

Diagnosis of silent myocardial ischemia during the staging of HIV-associated lymphoma with FDG PET/CT.

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Fasting 18F fluoro-deoxy-glucose positron emission tomography examinations are routinely performed for the staging of HIV-associated lymphomas. In addition to possible comorbidity factors, the chronic inflammation that occurs in HIV-infected patients together with the metabolic side effects of

Proteolytic enzyme activities in mononuclear cells and granulocytes of patients with various neurological disorders.

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Our studies showed a significantly increased neutral protease activity in mononuclear cells in patients with MS in relapse, active neuro-Behçet's disease, acute disseminated encephalomyelitis (ADEM) and polymyositis. Furthermore, in patients with ADEM there was a significant increase in the
Proteinases are widespread in neuronal or nonneuronal eukaryotic cells. They are suggested to play an important role in the turnover of proteins in neuronal perikaryon and axon, and digestion of the transported cytoskeletal proteins in synaptic terminals. We examined the effect of acrylamide (50

Experimentally derived structural constraints for amyloid fibrils of wild-type transthyretin.

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Transthyretin (TTR) is a largely β-sheet serum protein responsible for transporting thyroxine and vitamin A. TTR is found in amyloid deposits of patients with senile systemic amyloidosis. TTR mutants lead to familial amyloidotic polyneuropathy and familial amyloid cardiomyopathy, with an earlier age
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