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Repairing folding-defective α-sarcoglycan mutants by CFTR correctors, a potential therapy for limb-girdle muscular dystrophy 2D.

Carotti M, Marsolier J, Soardi M, Bianchini E, Gomiero C, Fecchio C, Henriques SF, Betto R, Sacchetto R, Richard I, Sandonà D.

Hum Mol Genet. 2018 Mar 15;27(6):969-984. doi: 10.1093/hmg/ddy013.


Ablation of S1P3 receptor protects mouse soleus from age-related drop in muscle mass, force, and regenerative capacity.

Bondì M, Germinario E, Pirazzini M, Zanetti G, Cencetti F, Donati C, Gorza L, Betto R, Bruni P, Danieli-Betto D.

Am J Physiol Cell Physiol. 2017 Jul 1;313(1):C54-C67. doi: 10.1152/ajpcell.00027.2017. Epub 2017 Apr 26.


Stat3 promotes mitochondrial transcription and oxidative respiration during maintenance and induction of naive pluripotency.

Carbognin E, Betto RM, Soriano ME, Smith AG, Martello G.

EMBO J. 2016 Mar 15;35(6):618-34. doi: 10.15252/embj.201592629. Epub 2016 Feb 22.


S1P3 receptor influences key physiological properties of fast-twitch extensor digitorum longus muscle.

Germinario E, Bondì M, Cencetti F, Donati C, Nocella M, Colombini B, Betto R, Bruni P, Bagni MA, Danieli-Betto D.

J Appl Physiol (1985). 2016 Jun 1;120(11):1288-300. doi: 10.1152/japplphysiol.00345.2015. Epub 2015 Dec 30.


Inhibition of ubiquitin proteasome system rescues the defective sarco(endo)plasmic reticulum Ca2+-ATPase (SERCA1) protein causing Chianina cattle pseudomyotonia.

Bianchini E, Testoni S, Gentile A, Calì T, Ottolini D, Villa A, Brini M, Betto R, Mascarello F, Nissen P, Sandonà D, Sacchetto R.

J Biol Chem. 2014 Nov 28;289(48):33073-82. doi: 10.1074/jbc.M114.576157. Epub 2014 Oct 6.


Unveiling the degradative route of the V247M α-sarcoglycan mutant responsible for LGMD-2D.

Bianchini E, Fanin M, Mamchaoui K, Betto R, Sandonà D.

Hum Mol Genet. 2014 Jul 15;23(14):3746-58. doi: 10.1093/hmg/ddu088. Epub 2014 Feb 23.


Effects of pleiotrophin overexpression on mouse skeletal muscles in normal loading and in actual and simulated microgravity.

Camerino GM, Pierno S, Liantonio A, De Bellis M, Cannone M, Sblendorio V, Conte E, Mele A, Tricarico D, Tavella S, Ruggiu A, Cancedda R, Ohira Y, Danieli-Betto D, Ciciliot S, Germinario E, Sandonà D, Betto R, Camerino DC, Desaphy JF.

PLoS One. 2013 Aug 28;8(8):e72028. doi: 10.1371/journal.pone.0072028. eCollection 2013.


Paracrine effects of IGF-1 overexpression on the functional decline due to skeletal muscle disuse: molecular and functional evaluation in hindlimb unloaded MLC/mIgf-1 transgenic mice.

Pierno S, Camerino GM, Cannone M, Liantonio A, De Bellis M, Digennaro C, Gramegna G, De Luca A, Germinario E, Danieli-Betto D, Betto R, Dobrowolny G, Rizzuto E, Musarò A, Desaphy JF, Camerino DC.

PLoS One. 2013 Jun 3;8(6):e65167. doi: 10.1371/journal.pone.0065167. Print 2014.


S1P2 receptor promotes mouse skeletal muscle regeneration.

Germinario E, Peron S, Toniolo L, Betto R, Cencetti F, Donati C, Bruni P, Danieli-Betto D.

J Appl Physiol (1985). 2012 Sep 1;113(5):707-13. doi: 10.1152/japplphysiol.00300.2012. Epub 2012 Jun 28.


Adaptation of mouse skeletal muscle to long-term microgravity in the MDS mission.

Sandonà D, Desaphy JF, Camerino GM, Bianchini E, Ciciliot S, Danieli-Betto D, Dobrowolny G, Furlan S, Germinario E, Goto K, Gutsmann M, Kawano F, Nakai N, Ohira T, Ohno Y, Picard A, Salanova M, Schiffl G, Blottner D, Musarò A, Ohira Y, Betto R, Conte D, Schiaffino S.

PLoS One. 2012;7(3):e33232. doi: 10.1371/journal.pone.0033232. Epub 2012 Mar 28.


Extracellular ATP signaling during differentiation of C2C12 skeletal muscle cells: role in proliferation.

Martinello T, Baldoin MC, Morbiato L, Paganin M, Tarricone E, Schiavo G, Bianchini E, Sandonà D, Betto R.

Mol Cell Biochem. 2011 May;351(1-2):183-96. doi: 10.1007/s11010-011-0726-4. Epub 2011 Feb 10.


Sorting receptor Rer1 controls surface expression of muscle acetylcholine receptors by ER retention of unassembled alpha-subunits.

Valkova C, Albrizio M, Röder IV, Schwake M, Betto R, Rudolf R, Kaether C.

Proc Natl Acad Sci U S A. 2011 Jan 11;108(2):621-5. doi: 10.1073/pnas.1001624108. Epub 2010 Dec 27.


Sphingosine 1-phosphate signaling is involved in skeletal muscle regeneration.

Danieli-Betto D, Peron S, Germinario E, Zanin M, Sorci G, Franzoso S, Sandonà D, Betto R.

Am J Physiol Cell Physiol. 2010 Mar;298(3):C550-8. doi: 10.1152/ajpcell.00072.2009. Epub 2009 Dec 30.


Eccentric contractions lead to myofibrillar dysfunction in muscular dystrophy.

Blaauw B, Agatea L, Toniolo L, Canato M, Quarta M, Dyar KA, Danieli-Betto D, Betto R, Schiaffino S, Reggiani C.

J Appl Physiol (1985). 2010 Jan;108(1):105-11. doi: 10.1152/japplphysiol.00803.2009. Epub 2009 Nov 12.


Sarcoglycanopathies: molecular pathogenesis and therapeutic prospects.

Sandonà D, Betto R.

Expert Rev Mol Med. 2009 Sep 28;11:e28. doi: 10.1017/S1462399409001203. Review.


High-frequency fatigue of skeletal muscle: role of extracellular Ca(2+).

Germinario E, Esposito A, Midrio M, Peron S, Palade PT, Betto R, Danieli-Betto D.

Eur J Appl Physiol. 2008 Oct;104(3):445-53. doi: 10.1007/s00421-008-0796-5. Epub 2008 Jun 17.


Inhibition of proteasome activity promotes the correct localization of disease-causing alpha-sarcoglycan mutants in HEK-293 cells constitutively expressing beta-, gamma-, and delta-sarcoglycan.

Gastaldello S, D'Angelo S, Franzoso S, Fanin M, Angelini C, Betto R, Sandonà D.

Am J Pathol. 2008 Jul;173(1):170-81. doi: 10.2353/ajpath.2008.071146. Epub 2008 Jun 5.


Trophic action of sphingosine 1-phosphate in denervated rat soleus muscle.

Zanin M, Germinario E, Dalla Libera L, Sandonà D, Sabbadini RA, Betto R, Danieli-Betto D.

Am J Physiol Cell Physiol. 2008 Jan;294(1):C36-46. Epub 2007 Oct 17.


Isoform switching in myofibrillar and excitation-contraction coupling proteins contributes to diminished contractile function in regenerating rat soleus muscle.

Esposito A, Germinario E, Zanin M, Palade PT, Betto R, Danieli-Betto D.

J Appl Physiol (1985). 2007 Apr;102(4):1640-8. Epub 2007 Jan 18.


Deficiency of alpha-sarcoglycan differently affects fast- and slow-twitch skeletal muscles.

Danieli-Betto D, Esposito A, Germinario E, Sandonà D, Martinello T, Jakubiec-Puka A, Biral D, Betto R.

Am J Physiol Regul Integr Comp Physiol. 2005 Nov;289(5):R1328-37. Epub 2005 Jul 7.


Ultrastructure of diaphragm from dystrophic alpha-sarcoglycan-null mice.

Jakubiec-Puka A, Biral D, Krawczyk K, Betto R.

Acta Biochim Pol. 2005;52(2):453-60.


The T-tubule membrane ATP-operated P2X4 receptor influences contractility of skeletal muscle.

Sandonà D, Danieli-Betto D, Germinario E, Biral D, Martinello T, Lioy A, Tarricone E, Gastaldello S, Betto R.

FASEB J. 2005 Jul;19(9):1184-6. Epub 2005 Apr 26.


Sphingosine 1-phosphate protects mouse extensor digitorum longus skeletal muscle during fatigue.

Danieli-Betto D, Germinario E, Esposito A, Megighian A, Midrio M, Ravara B, Damiani E, Libera LD, Sabbadini RA, Betto R.

Am J Physiol Cell Physiol. 2005 Jun;288(6):C1367-73. Epub 2005 Jan 19.


Characterization of the ATP-hydrolysing activity of alpha-sarcoglycan.

Sandonà D, Gastaldello S, Martinello T, Betto R.

Biochem J. 2004 Jul 1;381(Pt 1):105-12.


Effects of modulators of sarcoplasmic Ca2+ release on the development of skeletal muscle fatigue.

Germinario E, Esposito A, Megighian A, Midrio M, Betto R, Danieli-Betto D.

J Appl Physiol (1985). 2004 Feb;96(2):645-9.


Expression of sarco(endo)plasmic reticulum Ca(2+)-ATPase slow (SERCA2) isoform in regenerating rat soleus skeletal muscle depends on nerve impulses.

Germinario E, Esposito A, Midrio M, Betto R, Danieli-Betto D.

Exp Physiol. 2002 Sep;87(5):575-83.


Expression and functional characterization of SCaMPER: a sphingolipid-modulated calcium channel of cardiomyocytes.

Cavalli AL, O'Brien NW, Barlow SB, Betto R, Glembotski CC, Palade PT, Sabbadini RA.

Am J Physiol Cell Physiol. 2003 Mar;284(3):C780-90. Epub 2002 Nov 6.


Early changes of type 2B fibers after denervation of rat EDL skeletal muscle.

Germinario E, Esposito A, Megighian A, Midrio M, Biral D, Betto R, Danieli-Betto D.

J Appl Physiol (1985). 2002 May;92(5):2045-52.


Bupivacaine myotoxicity is mediated by mitochondria.

Irwin W, Fontaine E, Agnolucci L, Penzo D, Betto R, Bortolotto S, Reggiani C, Salviati G, Bernardi P.

J Biol Chem. 2002 Apr 5;277(14):12221-7. Epub 2002 Jan 14.


Apoptosis in the skeletal muscle of rats with heart failure is associated with increased serum levels of TNF-alpha and sphingosine.

Dalla Libera L, Sabbadini R, Renken C, Ravara B, Sandri M, Betto R, Angelini A, Vescovo G.

J Mol Cell Cardiol. 2001 Oct;33(10):1871-8.


Loss of dystrophin and some dystrophin-associated proteins with concomitant signs of apoptosis in rat leg muscle overworked in extension.

Biral D, Jakubiec-Puka A, Ciechomska I, Sandri M, Rossini K, Carraro U, Betto R.

Acta Neuropathol. 2000 Dec;100(6):618-26.


Expression and characterization of Edg-1 receptors in rat cardiomyocytes: calcium deregulation in response to sphingosine 1-phosphate.

Nakajima N, Cavalli AL, Biral D, Glembotski CC, McDonough PM, Ho PD, Betto R, Sandoná D, Palade PT, Dettbarn CA, Klepper RE, Sabbadini RA.

Eur J Biochem. 2000 Sep;267(18):5679-86.


Effects of fatigue on sarcoplasmic reticulum and myofibrillar properties of rat single muscle fibers.

Danieli-Betto D, Germinario E, Esposito A, Biral D, Betto R.

J Appl Physiol (1985). 2000 Sep;89(3):891-8.


The role of sphingolipids in the control of skeletal muscle function: a review.

Sabbadini RA, Danieli-Betto D, Betto R.

Ital J Neurol Sci. 1999 Dec;20(6):423-30. Review.


Functional roles of dystrophin and of associated proteins. New insights for the sarcoglycans.

Betto R, Biral D, Sandonà D.

Ital J Neurol Sci. 1999 Dec;20(6):371-9. Review.


Gender- and thyroid hormone-related transitions of essential myosin light chain isoform expression in rat soleus muscle during ageing.

Biral D, Ballarin F, Toscano I, Salviati G, Yu F, Larsson L, Betto R.

Acta Physiol Scand. 1999 Dec;167(4):317-23. Review.


Ecto-ATPase activity of alpha-sarcoglycan (adhalin).

Betto R, Senter L, Ceoldo S, Tarricone E, Biral D, Salviati G.

J Biol Chem. 1999 Mar 19;274(12):7907-12.


Early effects of denervation on sarcoplasmic reticulum properties of slow-twitch rat muscle fibres.

Midrio M, Danieli-Betto D, Megighian A, Betto R.

Pflugers Arch. 1997 Aug;434(4):398-405.


Sphingosylphosphocholine modulates the ryanodine receptor/calcium-release channel of cardiac sarcoplasmic reticulum membranes.

Betto R, Teresi A, Turcato F, Salviati G, Sabbadini RA, Krown K, Glembotski CC, Kindman LA, Dettbarn C, Pereon Y, Yasui K, Palade PT.

Biochem J. 1997 Feb 15;322 ( Pt 1):327-33.


Effects of age on sarcoplasmic reticulum properties and histochemical composition of fast- and slow-twitch rat muscles.

Danieli-Betto D, Betto R, Megighian A, Midrio M, Salviati G, Larsson L.

Acta Physiol Scand. 1995 May;154(1):59-64.


Involvement of ryanodine receptors in sphingosylphosphorylcholine-induced calcium release from brain microsomes.

Dettbarn C, Betto R, Salviati G, Sabbadini R, Palade P.

Brain Res. 1995 Jan 9;669(1):79-85.


Control of cardiac Ca2+ levels. Inhibitory actions of sphingosine on Ca2+ transients and L-type Ca2+ channel conductance.

McDonough PM, Yasui K, Betto R, Salviati G, Glembotski CC, Palade PT, Sabbadini RA.

Circ Res. 1994 Dec;75(6):981-9.


Maximum shortening velocity and coexistence of myosin heavy chain isoforms in single skinned fast fibres of rat skeletal muscle.

Bottinelli R, Betto R, Schiaffino S, Reggiani C.

J Muscle Res Cell Motil. 1994 Aug;15(4):413-9.


Modulation of cardiac sarcoplasmic reticulum ryanodine receptor by sphingosine.

Dettbarn CA, Betto R, Salviati G, Palade P, Jenkins GM, Sabbadini RA.

J Mol Cell Cardiol. 1994 Feb;26(2):229-42.


Localization of protein kinase C in skeletal muscle T-tubule membranes.

Salvatori S, Furlan S, Millikin B, Sabbadini R, Betto R, Margreth A, Salviati G.

Biochem Biophys Res Commun. 1993 Nov 15;196(3):1073-80.


Dystrophin is phosphorylated by endogenous protein kinases.

Luise M, Presotto C, Senter L, Betto R, Ceoldo S, Furlan S, Salvatori S, Sabbadini RA, Salviati G.

Biochem J. 1993 Jul 1;293 ( Pt 1):243-7.


Sphingosine is endogenous to cardiac and skeletal muscle.

Sabbadini R, McNutt W, Jenkins G, Betto R, Salviati G.

Biochem Biophys Res Commun. 1993 Jun 15;193(2):752-8.


Interaction of dystrophin with cytoskeletal proteins: binding to talin and actin.

Senter L, Luise M, Presotto C, Betto R, Teresi A, Ceoldo S, Salviati G.

Biochem Biophys Res Commun. 1993 Apr 30;192(2):899-904.


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