5 research outputs found
Diagnostic accuracy of patient interview items and clinical tests for cervical radiculopathy
- Author
- Publication venue
- Publication date
- 01/01/2021
- Field of study
The Importance of Government‐Sponsored Location Incentives for Distribution Facilities
- Author
- Publication venue
- 'Emerald'
- Publication date
- Field of study
FGF21 underlies a hormetic response to metabolic stress in methylmalonic acidemia
- Author
- Abu-Asab M.
- Andersson H.C.
- Andres-Mateos E.
- Chandler R.J.
- Cusmano-Ozog K.
- DiMauro S.
- Elkahloun A.G.
- Enns G.M.
- Epping M.W.
- Gagne J.
- Garone C.
- Girard C.L.
- Grunewald S.
- Hoffmann V.
- Kleiner D.E.
- Ktena Y.P.
- Li L.
- Li L.
- Manoli I.
- Ouattara B.
- Pass A.
- Schnermann J.
- Sloan J.L.
- Sysol J.R.
- Trivedi N.S.
- Tsokos M.G.
- Vandenberghe L.H.
- Venditti C.P.
- Vernon H.J.
- Wang C.
- Zerfas P.M.
- Publication venue
- 'American Society for Clinical Investigation'
- Publication date
- 01/01/2018
- Field of study
Methylmalonic acidemia (MMA), an organic acidemia characterized by metabolic instability and multiorgan complications, is most frequently caused by mutations in methylmalonyl-CoA mutase (MUT). To define the metabolic adaptations in MMA in acute and chronic settings, we studied a mouse model generated by transgenic expression of Mut in the muscle. Mut-/-;TgINS-MCK-Mut mice accurately replicate the hepatorenal mitochondriopathy and growth failure seen in severely affected patients and were used to characterize the response to fasting. The hepatic transcriptome in MMA mice was characterized by the chronic activation of stress-related pathways and an aberrant fasting response when compared with controls. A key metabolic regulator, Fgf21, emerged as a significantly dysregulated transcript in mice and was subsequently studied in a large patient cohort. The concentration of plasma FGF21 in MMA patients correlated with disease subtype, growth indices, and markers of mitochondrial dysfunction but was not affected by renal disease. Restoration of liver Mut activity, by transgenesis and liver-directed gene therapy in mice or liver transplantation in patients, drastically reduced plasma FGF21 and was associated with improved outcomes. Our studies identify mitocellular hormesis as a hepatic adaptation to metabolic stress in MMA and define FGF21 as a highly predictive disease biomarker
Nucleotide Biosynthetic Enzyme GMP Synthase Is a TRIM21-Controlled Relay of p53 Stabilization
- Author
- Adone Mohd-Sarip
- Aird
- Alice G.M. Bot
- B. Ashok Reddy
- Banin
- Bernatsky
- Bester
- Brand
- Brooks
- Brown
- C. Peter Verrijzer
- Cerami
- Chauhan
- Cummins
- Dick H.W. Dekkers
- Eltzschig
- Epping
- Faesen
- Frappier
- Grandinetti
- Hock
- Jackson
- Jan A. van der Knaap
- Jeroen A.A. Demmers
- John W.M. Martens
- Khoronenkova
- Kon
- Kon
- Kozhevnikova
- Kubbutat
- Lane
- Levine
- Li
- Li
- Maertens
- Maya
- Meulmeester
- Mieke A. Timmermans
- Nicholson
- Oke
- Ollmann
- Reichard
- Ringshausen
- Saridakis
- Sarkari
- Shieh
- Song
- Stommel
- Su
- Sullivan
- Tong
- van der Knaap
- van der Knaap
- Weber
- Xu
- Yoshimi
- Yuan
- Publication venue
- 'Elsevier BV'
- Publication date
- Field of study
Neurochemistry of Drug Abuse
- Author
- Acri J.
- Aghajanian G.K.
- Akiyama K.
- Akiyama K.
- Alburges M.
- Alburges M.E.
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- Alhassoon O.M.
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- Alleweireldt A.T.
- Almeida L.E.P.E.
- Aloyo V.
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- Ricaurte G.A.
- Ricaurte G.A.
- Rice M.E.
- Richardson N.
- Riddle E.L.
- Riegel A.C.
- Ritz M.
- Ritz M.C.
- Ritz M.C.
- Ritz M.C.
- Robbins T.W.
- Robbins T.W.
- Roberts D.
- Roberts D.
- Roberts D.C
- Roberts D.C.
- Roberts D.C.
- Roberts D.C.
- Robertson G.S.
- Robinson D.L.
- Robinson T.E.
- Robinson T.E.
- Robinson T.E.
- Robinson T.E.
- Robinson T.E.
- Robinson T.E.
- Robinson T.E.
- Robinson T.E.
- Robinson T.E.
- Robledo P.
- Rocha B.A.
- Rocha B.A.
- Rocher C.
- Rocio M.
- Rocio M.
- Rockhold R.
- Rockhold R.W
- Romero L.
- Rommelspacher H.
- Rose J.E.
- Rosin A.
- Rostene W.
- Rothman R.
- Rothman R.
- Rothman R.
- Rothman R.B.
- Rothman R.B.
- Rothman R.B.
- Rothman R.B.
- Rothman R.B.
- Rudnick G.
- Rukstalis M.
- Rush R.
- Russell M.A
- Russell V.A.
- Ruttenber A.J.
- Saal D.
- Sabol S.Z.
- Sakurai-Yamashita Y.
- Salminen O.
- Salminen O.
- Samson H.H.
- Samuel D.
- Sanchez C.J.
- Sanchez-Ramos J.
- Satel S.
- Satel S.
- Satel S.L.
- Satel S.L.
- Savelieva K.V.
- Scanzello C.R.
- Schechter M.D.
- Scheffel U.
- Scheffel U.
- Scheffel U.
- Schenk S.
- Schenk S.
- Schenk S.
- Schenk S.
- Schenk S.
- Schenk S.
- Schenk S.
- Schievelbein H.
- Schifano F.
- Schiffer W.K.
- Schilstrom B.
- Schmidt C.J.
- Schmidt C.J.
- Schmidt C.J.
- Schmidt C.J.
- Schmidt C.J.
- Schmidt C.J.
- Schneider N.G.O.R.
- Schoemaker H.
- Schroeder B.E.
- Schuckit M.A.
- Schuldiner S.
- Schultz W
- Schwartz K.
- Seiden L.S.
- Seiden L.S.
- Seiden L.S.
- Sekine Y.
- Self D
- Self D.
- Self D.W.
- Self D.W.
- Self D.W.
- Sellings L.H.
- Series H.G.
- Series H.G.
- Sershen H.
- Sesack S.R.
- Setola V.
- Shank R.P.
- Shankaran M.
- Shankaran M.
- Sharkey J.
- Shaya E.K.
- Shaywitz A.J.
- Shiah I.S.Y.L
- Shier W.T.
- Shiffman S.J.J.
- Shimada S.
- Shimosato K.
- Shippenberg T.
- Shippenberg T.
- Shippenberg T.
- Shippenberg T.
- Shoaib M.
- Shoaib M.
- Shoaib M.
- Shoblock J.R.
- Shoptaw S.
- Silva A.J.
- Simantov R.
- Simonin F.
- Sizemore G.M.
- Slikker W. Jr.
- Slusher B.
- Smith J.E.
- Smith R.C.
- Smolka M.N.
- Snyder G.L.
- Snyder S.H.
- Snyder S.J.
- Sokoloff P.
- Sonsalla P.K.
- Sora I.
- Sora I.
- Sorg B.A.
- Spampinato U.
- Spanagel R.
- Spanagel R.
- Spangler R.
- Spangler R.
- Spanos L.J.
- Sparks J.A.
- Sparrow D.
- Spealman R
- Spealman R.
- Spielwoy C.
- Spitz M.R.
- Sprague J.E.
- Sprouse J.S.
- Spyraki C.
- Spyraki C.
- Srisurapanont M.
- Staley J.
- Staley J.
- Staley J.
- Staley J.
- Staley J.
- Staley J.
- Staley J.
- Staley J.K.
- Staley J.K.
- Staley J.K.
- Staley J.K.
- Staley J.K.
- Stathis M.
- Stathis M.
- Steele T.D.
- Stefanski R.
- Stefanski R.
- Stefanski R.
- Stegmayr B.
- Steinbusch H.W.
- Steinpreis R.E.
- Stephens B.G.
- Stephens B.G.
- Stone D.M.
- Streeter C.C.
- Striplin C.D.
- Stromberg M.F.
- Strombom U.H.
- Suaad-Chagny M.F.
- Suemaru J.
- Sullivan P.F.
- Sulzer D.
- Sulzer D.
- Sulzer D.
- Sutherland G.
- Svingos A.L.
- Sweatt J.D
- Sweatt J.D
- Sweetnam P.
- Switzer R.C. III
- Szumlinski K.K.
- Takahashi S.
- Takaki M.
- Takaki M.
- Tan A.Y.
- Tancer M.
- Tapper A.R.
- Tella S
- Tennant F.
- Terwilliger R.Z.
- Tessari M.
- This
- Thomas G.M.
- Tolliver B.K.
- Tong J.
- Total
- Trujillo K.
- Trulson M.E.
- Tsukada H.
- Tucker G.J.
- Turek J.W.
- Turgeon S.M.
- Tzschentke T.M
- Ujike H.
- Ujike H.
- Ujike H.
- Ujike H.
- Ujike H.
- Ukai M.
- Ungerstedt U.
- Unterwald E.
- Unterwald E.
- Unterwald E.M
- Unterwald E.M.
- Uramura K.
- Vailati S.
- Valjent E.
- Valjent E.
- Valjent E.
- Van de Kar L.D.
- Vandenbergh D.J.
- Vaughan R.A.
- Vignon J.
- Villegier A.S.
- Villemagne V.
- Villemagne V.
- Vinklerova J.
- Volkow N.
- Volkow N.
- Volkow N.
- Volkow N.
- Volkow N.D.
- Volkow N.D.
- Volkow N.D.
- Volkow N.D.
- Volkow N.D.
- Volkow N.D.
- Volkow N.D.
- Volkow N.D.
- Volkow N.D.
- Vollenweider F.X.
- Vollm B.A.
- Volz T.J.
- Wachtel H
- Wagner G.C.
- Wagner G.C.
- Walaas S.I.
- Wallace D.
- Walsh S.
- Walsh S.L.
- Walters C.L.
- Walters C.L.
- Wang F.
- Wang G.J.
- Wang G.J.
- Wang X.
- Wang X.
- Weddington W.W.
- Weintraub J.M.
- Weiss F.
- Wesson D.R
- West G.B.
- Westfall T.C.
- Wetli C.V.
- Wetli C.V.
- White C.R.
- White F.
- Whiteaker P.
- Wilson J.
- Wilson J.
- Wilson J.M.
- Wilson J.M.
- Wilson L.D.
- Wilson M.
- Winhusen T.M.
- Wise R.A
- Witkin J
- Witkin J.M.
- Witkin J.M.
- Wolf M.
- Wolf M.E.
- Wollemann M.
- Wong A.H.C.
- Wong G.
- Wonnacott S
- Wonnacott S.
- Wood M.D.
- Woodward J.J.
- Wooltorton J.R.
- Woolverton W.
- Woolverton W.L.
- Woolverton W.L.
- Worsley J.N.
- Worsley J.N.
- Wrona M.Z.
- Wu D.
- Wu J.
- Wu X.
- Xi Z.X.
- Xi Z.X.
- Xu Z.
- Yacoubian G.S. Jr.
- Yamamoto B.K.
- Yamamoto B.K.
- Yamashita H.
- Yan Q.S.
- Yan Z.
- Yeghiayan S.K.
- Yim H.J.
- Yokel R.A.
- Yoshimoto K.
- Yui K.
- Yui K.
- Yui K.
- Zachariou V.
- Zaczek R.
- Zaczek R.
- Zaczek R.
- Zeidonis D.
- Zeigler S.
- Zetterstrom T.
- Zhang D.
- Zhang H.
- Zhang X.
- Zhang X.Y.
- Zigmond M.J.
- Zimanyi I.
- Zito K.A.
- Zito K.A.
- Publication venue
- 'Informa UK Limited'
- Publication date
- Field of study