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A Mutation in the TRPC6 Cation Channel Causes Familial Focal Segmental Glomerulosclerosis
The proline-to-glutamine substitution at position 112, which occurs in a highly conserved region of the protein, enhances TRPC6-mediated calcium signals in response to agonists such as angiotensin II and appears to alter the intracellular distribution of TRPC 6 protein. Expand
Targeted inactivation of the sodium‐calcium exchanger (Ncx1) results in the lack of a heartbeat and abnormal myofibrillar organization
It is demonstrated that Ncx‐1 is a Ca2+‐ gene that is essential for normal cardiomyocyte development and function and may serve as an animal model for functionally related human congenital heart defects. Expand
Role of cardiac neural crest cells in cardiovascular development.
Evidence indicates that poor viability is from impaired cardiac excitation-contraction coupling and contractile function at the myocyte level, and the continued study of experimental and genetically defined models with neural crest-associated heart defects will prove useful in identifying the common pathways by which the neural crest contributes to normal heart development. Expand
Localization, Macromolecular Associations, and Function of the Small Heat Shock–Related Protein HSP20 in Rat Heart
HSP20 is associated with &agr;B-crystallin, possibly at the level of the actin sarcomere, and increases myocyte shortening rate through increases in calcium uptake and more rapid lengthening. Expand
Cardiac arterial pole alignment is sensitive to FGF8 signaling in the pharynx.
The results indicate that the secondary heart field myocardium is particularly sensitive to FGF8 signaling for normal conotruncal development, and further, that cardiac neural crest cells modulate F GF8 signaling in the caudal pharynx. Expand
Spectral-domain phase microscopy.
Broadband interferometry is an attractive technique for the detection of cellular motions because it provides depth-resolved phase information via coherence gating. We present a phase-sensitiveExpand
A novel role for cardiac neural crest in heart development.
The results indicate a novel role for neural crest cells in myocardial maturation, as evidenced by normal expression of fibrillin-2 protein (JB3 antigen) and normal formation of cushion mesenchyme and trabeculae in the absence of cardiac neural crest. Expand
Neural crest is involved in development of abnormal myocardial function.
An important role for the neural crest in the development of normal myocardial function is indicated, and the first demonstration of impaired excitation-contraction coupling in a genetically-defined embryonic mammalian model of a cardiac structural defect is demonstrated. Expand
Myocardial volume and organization are changed by failure of addition of secondary heart field myocardium to the cardiac outflow tract
The decreased volume and number of myocardial cells in the heart tube after neural crest ablation most likely reflects the amount of mycardium added by the secondary heart field. Expand
A time-dependent and voltage-sensitive K+ current in single cells from frog atrium
The whole-cell patch-clamp technique is used to record the delayed outward K+ current, IK, in single cells dissociated from frog atrium, and this current has been characterized according to its steady state activation, kinetics, and ion transfer function. Expand