Integrins engage in bidirectional signaling. Fibrinogen binding to integrins is regulated by inside-out signals initiated by agonist receptors, a process often called “integrin activation.” In turn, ligandbound integrins transduce outside-in signals to regulate cellular responses, among them cytoskeletal reorganization. In platelets, integrin aIIbb3 bidirectional signaling is required for effic...

Adhesion of platelets to extracellular matrix via von Willebrand factor (vWF) and activation of platelets by thrombin are critical steps in hemostasis. Glycoprotein (GP) V is a component of the GPIb-V-IX complex, the platelet receptor for vWF. GPV is also cleaved by thrombin. Deficiency of GPIb or GPIX results in Bernard-Soulier syndrome (BSS), a bleeding disorder in which platelets are giant a...

Darier disease(DD) is a genetic disorder characterized by impaired intercellular adhesion and aberrant differentiation of keratinocytes, leading to hyperkeratotic papules epidermal erosions. While mutations underlying DD are known target SERCA2, an endoplasmic reticulum(ER) calcium pump, current therapies for this chronic, painful skin limited some, such as oral retinoids, may pose significant ...

Vitamin B12 deficiency has long been known to present with various neurological manifestations, but only rarely presents as movement disorders, especially in adults. We the case of a 30-year-old vegan male presenting tremors on both legs when standing which was relieved by vitamin supplementation. To best our knowledge, this is first documented slow orthostatic tremor or pseudo-orthostatic caus...

to the editor: Ramaekers et al. (May 12 issue)1 report 28 patients with the cerebral folate deficiency syndrome characterized by decreased levels of 5-methyltetrahydrofolate (5MTHF) in the cerebrospinal fluid caused by autoantibodies’ blocking of folate transport into the brain. The patients benefited from folinic acid supplementation. The clinical features of this syndrome fit with other neuro...