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cookies. View All num of num Close (Esc) BCAA Engaged Employer Overview Overview 56 Reviews 33 Salaries 9 Interviews itunes sync error 54 windows 10 14 Benefits 58 Jobs More Photos Follow Add a Review Follow Add a Review BCAA Reviews Updated September 16, 2016 54 reviews Filter Filter Full-time Part-time Job Title Location Location Worldwide Australia
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- All Cities – Sydney Area Canada - All Cities – Vancouver, BC Area (includes HQ) – Victoria, BC Area Employment Status Intern Freelance Contract Part-time Full-time Current Employee Clear All Apply Filters Clear All BCAA 54 2.8 Star Star Star Star Star [REGULAR, PART_TIME, UNKNOWN] true Rating Trends Rating Trends Recommend to a friend Approve of CEO Shom Sen 4 Ratings Close what is itunes error -39 Your trust is our top concern, so companies can't alter or remove reviews. 54 Employee Reviews Sort: Popular Rating Date Pros The pay and benefits are above average (in 4 reviews) nice work environment they encourage learning and the education good medical plan (in 5 reviews) Great people to work with who all share the same values (in 3 reviews) I am looking forward to see what the future of BCAA holds with our new CEO (in 3 reviews) There are some amazingly smart people that work there but unfortunatly it is about who you know (in 2 reviews) Cons With new CEO, change was inevitable (in 4 reviews) Management type people will have no chance unless you are willing to get a MBA and become a yes man (in 3 reviews) can't believe how many people they let go of or forced out in the 8 years I worked there,some awesome talented people including myself forced out (in 2 reviews) Not too friendly with new employees (not enough attention) (in 2 reviews) More Pros and Cons Sep 16, 2016 "Insurance Advisor" Star Star Star Star
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ketoacidosis and promotes metabolic homeostasis in MSUDDavis S. Sonnet1 n1, Monique N. O’Leary1, 2 n1, Mark A. Gutierrez3, Steven M. Nguyen1, Samiha Mateen1, Yuehmei Hsu1, Kylie P. Mitchell1, Antonio J. Lopez4, Jerry Vockley5, Brian K. Kennedy1& Arvind Ramanathan1Scientific Reports 6, Articlenumber:28775 http://www.nature.com/articles/srep28775 (2016)doi:10.1038/srep28775Download CitationMetabolismSmall moleculesReceived:04 March 2016Accepted:08 June 2016Published online:04 July 2016AbstractMaple Syrup Urine Disease (MSUD) is an inherited disorder caused by the dysfunction in the branched chain keto-acid dehydrogenase (BCKDH) enzyme. This leads to buildup of branched-chain keto-acids (BCKA) and branched-chain amino acids (BCAA) in body fluids (e.g. keto-isocaproic acid from the BCAA leucine), leading to numerous clinical features including a less error 54 understood skeletal muscle dysfunction in patients. KIC is an inhibitor of mitochondrial function at disease relevant concentrations. A murine model of intermediate MSUD (iMSUD) shows significant skeletal muscle dysfunction as by judged decreased muscle fiber diameter. MSUD is an orphan disease with a need for novel drug interventions. Here using a 96-well plate (liquid chromatography- mass spectrometry (LC-MS) based drug-screening platform error 54 windows we show that Metformin, a widely used anti-diabetic drug, reduces levels of KIC in patient-derived fibroblasts by 20–50%. This Metformin-mediated effect was conserved in vivo; Metformin-treatment significantly reduced levels of KIC in the muscle (by 69%) and serum (by 56%) isolated from iMSUD mice, and restored levels of mitochondrial metabolites (e.g. AMP and other TCA). The drug also decreased the expression of mitochondrial branched chain amino transferase (BCAT) which produces KIC in skeletal muscle. This suggests that Metformin can restore skeletal muscle homeostasis in MSUD by decreasing mitochondrial KIC production.IntroductionMaple Syrup Urine Disease (MSUD) is an inherited disorder in branched-chain amino acid (BCAA) catabolism caused by mutations in genes that encode the components of the branched chain keto-acid dehydrogenase (BCKDH) complex, which catalyzes the first irreversible step in BCAA catabolism1,2,3. BCKDH dysfunction results in the accumulation of the keto-acids ketoisocaproic acid (KIC) from leucine, ketoisovaleric acid (KIV) from valine and ketomethylvaleric acid (KMV) from isoleucine, produced by the upstream enzymes cytosolic branched-chain amino acid transaminase (BCAT-1) and mitochondrial BCAT-2 (Fig. 1a). BCAT-2 is the major isoform expressed in skeletal muscle, and re
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