User profiles for Marino DiFranco

Marino DiFranco

Adjunct Professor
Verified email at mednet.ucla.edu
Cited by 1137

DNA transfection of mammalian skeletal muscles using in vivo electroporation

M DiFranco, M Quinonez, J Capote… - JoVE (Journal of Visualized …, 2009 - jove.com
A growing interest in cell biology is to express transgenically modified forms of essential
proteins (eg fluorescently tagged constructs and/or mutant variants) in order to investigate their …

The action potential‐evoked sarcoplasmic reticulum calcium release is impaired in mdx mouse muscle fibres

CE Woods, D Novo, M DiFranco… - The Journal of …, 2004 - Wiley Online Library
The mdx mouse, a model of the human disease Duchenne muscular dystrophy, has skeletal
muscle fibres which display incompletely understood impaired contractile function. We …

Na,K-ATPase α2 activity in mammalian skeletal muscle T-tubules is acutely stimulated by extracellular K+

M DiFranco, H Hakimjavadi, JB Lingrel… - Journal of General …, 2015 - rupress.org
The Na,K-ATPase α2 isoform is the predominant Na,K-ATPase in adult skeletal muscle and
the sole Na,K-ATPase in the transverse tubules (T-tubules). In quiescent muscles, the α2 …

Stac3 enhances expression of human CaV1.1 in Xenopus oocytes and reveals gating pore currents in HypoPP mutant channels

F Wu, M Quinonez, M DiFranco… - Journal of General …, 2018 - rupress.org
Mutations of Ca V 1.1, the pore-forming subunit of the L-type Ca 2+ channel in skeletal
muscle, are an established cause of hypokalemic periodic paralysis (HypoPP). However, …

Propagation in the transverse tubular system and voltage dependence of calcium release in normal and mdx mouse muscle fibres

CE Woods, D Novo, M DiFranco… - The Journal of …, 2005 - Wiley Online Library
Using a two‐microelectrode voltage clamp technique, we investigated possible mechanisms
underlying the impaired excitation–contraction coupling in skeletal muscle fibres of the mdx …

The distinct role of the four voltage sensors of the skeletal CaV1.1 channel in voltage-dependent activation

…, J Wier, F Wu, M Quinonez, M DiFranco… - Journal of General …, 2021 - rupress.org
Initiation of skeletal muscle contraction is triggered by rapid activation of RYR1 channels in
response to sarcolemmal depolarization. RYR1 is intracellular and has no voltage-sensing …

Pathogenity of some limb girdle muscular dystrophy mutations can result from reduced anchorage to myofibrils and altered stability of calpain 3

N Ermolova, E Kudryashova, M DiFranco… - Human molecular …, 2011 - academic.oup.com
Calpain 3 (CAPN3) is a muscle-specific, calcium-dependent proteinase that is mutated in Limb
Girdle Muscle Dystrophy type 2A. Most pathogenic missense mutations in LGMD2A affect …

Excitation-contraction coupling alterations in mdx and utrophin/dystrophin double knockout mice: a comparative study

J Capote, M DiFranco… - American Journal of …, 2010 - journals.physiology.org
The double knockout mouse for utrophin and dystrophin (utr −/− /mdx) has been proposed
to be a better model of Duchenne Muscular Dystrophy (DMD) than the mdx mouse because …

Chloride currents from the transverse tubular system in adult mammalian skeletal muscle fibers

M DiFranco, A Herrera, JL Vergara - Journal of General Physiology, 2011 - rupress.org
Chloride fluxes are the main contributors to the resting conductance of mammalian skeletal
muscle fibers. ClC-1, the most abundant chloride channel isoform in this preparation, is …

[HTML][HTML] Effects of membrane depolarization and changes in extracellular [K+] on the Ca2+ transients of fast skeletal muscle fibers. Implications for muscle fatigue

…, F González, C Morgado-Valle, M DiFranco - Journal of muscle …, 2010 - Springer
Repetitive activation of skeletal muscle fibers leads to a reduced transmembrane K + gradient.
The resulting membrane depolarization has been proposed to play a major role in the …