Efficient synaptic transmission at the neuromuscular junction (NMJ) requires the topological maturation of the postsynaptic apparatus from an oval acetylcholine receptor (AChR)-rich plaque into a complex pretzel-shaped array of branches. However, compared to NMJ formation very little is known about the mechanisms that regulate NMJ maturation. Recently the process of in vivo transformation from ...

Captive farming of alpine musk deer (Moschus sifanicus) in China has been used for conservation and harvesting of musk since the mid 1950s. Despite this long history, management practices and captive breeding have been primarily based on trial and error due to lack of behavioral and ecological information about this vulnerable species. Understanding behavioral patterns plays a vital part in det...

Muscle-specific kinase (MuSK) belongs to the nicotinic acetylcholine receptor complex which is targeted by pathogenic autoantibodies causing Myasthenia gravis. While up to 95% of patients with generalized Myasthenia gravis were shown to be positive for acetylcholine receptor-specific autoantibodies, up to 70% of the remaining patients develop autoantibodies against MuSK. Discrimination of the a...

BACKGROUND AND PURPOSE Responses to repetitive nerve stimulation (RNS) in patients with muscle-specific tyrosine kinase (MuSK) antibody (Ab)-positive myasthenia gravis (MG) vary depending on the muscles tested. We analyzed the RNS responses of limb and facial muscles in MuSK-Ab-positive and acetylcholine receptor (AChR)-Ab-negative MG (MuSK MG) and MuSK-Ab-negative and AChR-Ab-negative [double-...

During synaptogenesis at the neuromuscular junction, a neurally released factor, agrin, causes the clustering of acetylcholine receptors (AChRs) in the muscle membrane beneath the nerve terminal. Agrin acts through a specific receptor which is thought to have a receptor tyrosine kinase, MuSK, as one of its components. In agrin-treated muscle cells, both MuSK and the AChR become tyrosine phospho...

In skeletal muscle fibers, neural agrin can direct the accumulation of acetylcholine receptors (AChR) and transcription of AChR subunit genes from the subsynaptic nuclei. Although the receptor tyrosine kinase MuSK is required for AChR clustering, it is less clear whether MuSK regulates gene transcription. To elucidate the role of MuSK in these processes, we constructed a constitutively active M...

Musk deer are highly important as a medicinal species that are severely exploited throughout their range of occurrence due to the medicinal value of the musk produced only by the male individuals. Methods used for studying the populations and distributions of other ungulates do not work well with musk deer and the presence of a unified methodology for studying musk deer appear to be lacking wor...

Musk ambrette, musk ketone and musk xylene have a long history of use as fragrance ingredients, although musk ambrette is no longer used in fragrances. As part of the review of the safety of these uses, it is important to consider the systemic exposure that results from these uses. Since the primary route of exposure to fragrances is on the skin, dermal doses of carbon-14 labelled musk ambrette...

OBJECTIVES To compare the clinical and electrophysiological features of myasthenia gravis (MG) patients with (seropositive) or without (seronegative) antibodies to acetylcholine receptor. To investigate whether antibodies to muscle specific kinase (MuSK) and ryanodine receptor (RyR) are associated with particular features. METHODS Clinical profiles and single fibre electromyography (SFEMG) in...

OBJECTIVE To characterize B-cell subsets in patients with muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG). METHODS In accordance with Human Immunology Project Consortium guidelines, we performed polychromatic flow cytometry and ELISA assays in peripheral blood samples from 18 patients with MuSK MG and 9 healthy controls. To complement a B-cell phenotype assay that evaluated matu...