In vitro epidermal cell proliferation in rat skin plugs.

Full-thickness skin plugs from immature and adult rats have been shown to incorporate -3H-thymidine in vitro in a semisynchronous fashion for up to 54 hr. DNA synthesis is minimal at 16 hr post sacrifice but increases again at 24 and 48 hr, and cells in S phase at 48 hr have passed through at least one mitosis in vitro (between 32 and 39 hr). Advantage can be taken of this semisynchronous burst of DNA synthetic activity to test the effects of potential inhibitors of skin cell proliferation, and to determine at which phase of the cell cyclic these inhibitors act. High concentrations of epinephrine or isoproterenol (10--5 M) are required to cause inhibition but the effect is specific for the G2 phase of the cell cycle. Propranolol does not demonstrate beta-antagonism in this system, since it is a very potent inhibitor acting in G1 phase. Dibutyryl cycle AMP and theophylline have two effects: one is a specific inhibitory action on cells in the G2 phase and the second is a short-term action limiting thymidine uptake by cells in S phase without affecting the transition of cells from G1 to S.     

斩龙剑中新苯丙素甙的结构鉴定

从玄参科植物斩龙剑(Veronivastrum sibirica (L.)Pennell.)的根部分得九种化学成分,分别为斩龙剑甙A(sibiriosideA,Ⅰ)斩龙剑甙B(sibiriosideB,Ⅱ),异阿魏酸(isoferulicacid,Ⅲ),6-(4-methoxyferulpol)mioporoside(Ⅳ),scrophularioside(Ⅴ),6-O-阿魏酰梓醇(6-O-trans-feruloylcatalpol,Ⅵ),阿魏酸(ferulicacid,Ⅶ),胡萝卜甾醇(daucosterol,Ⅷ)和β-谷甾醇(Ⅸ)。其中斩龙剑甙A和斩龙剑甙B为新苯丙素甙化合物,应用波谱分析和理化数据测定,确定了它们的结构。     

Biallelic variants in CEP164 cause a motile ciliopathy-like syndrome

Ciliopathies may be classed as primary or motile depending on the underlying ciliary defect and are usually considered distinct clinical entities. Primary ciliopathies are associated with multisystem syndromes typically affecting the brain, kidney, and eye, as well as other organ systems such as the liver, skeleton, auditory system, and metabolism. Motile ciliopathies are a heterogenous group of disorders with defects in specialised motile ciliated tissues found within the lung, brain, and reproductive system, and are associated with primary ciliary dyskinesia, bronchiectasis, infertility and rarely hydrocephalus. Primary and motile cilia share defined core ultra-structures with an overlapping proteome, and human disease phenotypes can reflect both primary and motile ciliopathies. CEP164 encodes a centrosomal distal appendage protein vital for primary ciliogenesis. Human CEP164 mutations are typically described in patients with nephronophthisis-related primary ciliopathies but have also been implicated in motile ciliary dysfunction. Here we describe a patient with an atypical motile ciliopathy phenotype and biallelic CEP164 variants. This work provides further evidence that CEP164 mutations can contribute to both primary and motile ciliopathy syndromes, supporting their functional and clinical overlap, and informs the investigation and management of CEP164 ciliopathy patients.