Recently, epidemiological studies on the etiology of peripheral neuropathies have revealed that hyperlipidemia is a novel risk factor. Plasma lipid levels were confirmed to be associated with the incidence of many peripheral neuropathies including axonal distal polyneuropathy, vision and hearing loss, motor nerve system lesions and sympathetic nerve system dysfunction. Moreover, different lipid...

β-Site amyloid precursor protein (APP) cleaving enzyme 1 (BACE1) is an aspartyl protease best known for its role in generating the amyloid-β peptides that are present in plaques of Alzheimer's disease. BACE1 has been an attractive target for drug development. In cultured embryonic neurons, BACE1-cleaved N-terminal APP is further processed to generate a fragment that can trigger axonal degenerat...

background: diabetes mellitus is the most common metabolic disorder with many complications such as neuropathic pain which relief from it is a dilemma. objectives: to investigate the effect of hydroethanolic extract of rubus fruticosus on neuropathic pain in diabetic rats. materials and methods: in this experimental case –control study, forty eight male wistar rats (250±20 g) were divided rando...

Carpal tunnel syndrome (CTS) is the most well-known and frequent form of median nerve entrapment, and accounts for 90% of all entrapment neuropathies. Entrapment neuropathies of the ulnar nerve are relatively common with ulnar neuropathy at the elbow more prevalent than ulnar neuropathy at the wrist. The diagnosis of ulnar neuropathy at the elbow is usually confirmed in a relatively straight – ...

To assess left ventricular diastolic filling in patients with amyloid heart disease 12 patients with familial amyloid polyneuropathy and 15 normal subjects were studied by pulsed Doppler echocardiography. None of the patients had clinical evidence of overt heart disease or restrictive cardiomyopathy and only two of them showed ventricular wall thickening. The peak flow velocity of rapid diastol...

Background: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is a fatal, chronic, progressive disease. It is a rare hereditary amyloidosis, which manifests as a sensorimotor neuropathy and autonomic dysfunction. It begins during adulthood. Aims and Methods: Our aim is to evaluate psychopathological dimensions in a population attending a consultation center for TTR-FAP. Two hundred and ni...

BACKGROUND Familial amyloid polyneuropathy related to transthyretin gene (TTR-FAP) is a life-threatening disease transmitted as an autosomal dominant trait. Val30Met mutation accounts for the majority of the patients with large endemic foci especially in Portugal, Sweden and Japan. However, more than one hundred other mutations have been described worldwide. A great phenotypic variability among...

Mutated transthyretin (TTR) causes familial amyloid polyneuropathy, a neurodegenerative disorder characterized by TTR deposition in the peripheral nervous system (PNS). The origin/reason for TTR deposition in the nerve is unknown. Here we demonstrate that both endogenous mouse TTR and TTR injected intravenously have access to the mouse sciatic nerve. We previously determined that in the absence...

Transthyretin familial amyloid polyneuropathy (TTR-FAP) is an autosomal dominant disorder caused by the extracellular deposition of insoluble amyloid fibrils in the endoneurium. TTR-FAP occurs as a result of transthyretin (TTR) gene mutation, and more than 100 TTR mutation types are recognized [1]. It is a highly disabling and life-threatening disease, characterized by progressive sensorimotor ...