One of the first people in the world to perform live using the all-important ALK2
technology, the upcoming gig will see Ian create a series of multi-layered tracks - featuring guest vocals from folk singer Kitty MacFar-Turn to Page 40 From Page 39 lane, who is also playing a support slot - and there won't be a looping pedal in sight.
FOP is caused by a mutation in the gene for ALK2
, which is known as ACVR1, that causes hypersensitivity to certain bone morphogenetic proteins and a neomorphic response to activins.
Wrana, "Specific activation of Smad1 signaling pathways by the BMP7 type I receptor, ALK2
," Journal of Biological Chemistry, vol.
The FOP-iPSC lines containing ALK2
mutation displayed decreased differentiation efficiency into bone-forming progenitors and reduced expression of VEGF receptor 2 in differentiated endothelial cells.
Scheideler, "MicroRNA-30c promotes human adipocyte differentiation and co-represses PAI-1 and ALK2
," RNA biology, vol.
A nivel de superficie celular, el dimero de BMP se une al receptor tipo I (activin receptor-like kinase; ALK2
, ALK3, o ALK6) y al receptor tipo II (BMPR-II, ActR-IIA, o ActRIIB) para la formacion de complejos heteromericos.
The company stated that the two compounds that received orphan drug designation are small-molecule kinase inhibitors designed to selectively block a specific member of the bone morphogenetic protein (BMP) type-I receptor family, ALK2
. The seven members of the BMP type-I receptor family, activin receptor-like kinase (ALK) 1-7, play critical roles in human development and physiology.
It has been found that the BMPs are expressed in a specific time and place in order to regulate the growth of craniofacial elements derived from the neural crest, and that Nog union alters this growth through via binding to type I receptor Alk2
using the Wnt pathway, which results in cleft lip and palate and mandibular hypoplasia (74).
Specific activation of Smad1 signaling pathways by the BMP7 type I receptor, ALK2
. J Biol Chem 1998; 273: 25628-25636, doi: 10.1074/jbc.273.40.25628.
One possible explanation is that BMP7 preferentially acts through Alk2
(a stimulatory pathway), whereas BMP2/4 preferentially acts through Alk3 (an inhibitory pathway).