Class information for:
Level 1: MAPLE SYRUP URINE DISEASE//BRANCHED CHAIN ALPHA KETO ACID DEHYDROGENASE COMPLEX//BRANCHED CHAIN ALPHA KETOACID DEHYDROGENASE

Basic class information
Class id #P Avg. number of
references		 Database coverage
of references
9764 1122 30.8 58%


Bar chart of Publication_year

Last years might be incomplete

Hierarchy of classes
The table includes all classes above and classes immediately below the current class.


Cluster id Level Cluster label #P
8 4 CARDIAC & CARDIOVASCULAR SYSTEM//PERIPHERAL VASCULAR DISEASES//CARDIOL 1276458
257 3       JOURNAL OF PARENTERAL AND ENTERAL NUTRITION//ENTERAL NUTRITION//NUTRITION & DIETETICS 44865
1056 2             GLUTAMINE//MAPLE SYRUP URINE DISEASE//LEUCINE 10038
9764 1                   MAPLE SYRUP URINE DISEASE//BRANCHED CHAIN ALPHA KETO ACID DEHYDROGENASE COMPLEX//BRANCHED CHAIN ALPHA KETOACID DEHYDROGENASE 1122

Terms with highest relevance score


rank Term termType Chi square Shr. of publ. in
class containing
term		 Class's shr. of
term's tot. occurrences		 #P with
term in
class
1 MAPLE SYRUP URINE DISEASE authKW 2816531 12% 75% 138
2 BRANCHED CHAIN ALPHA KETO ACID DEHYDROGENASE COMPLEX authKW 593422 2% 84% 26
3 BRANCHED CHAIN ALPHA KETOACID DEHYDROGENASE authKW 544901 3% 69% 29
4 BRANCHED CHAIN AMINO ACIDS authKW 361611 9% 13% 103
5 MSUD authKW 314887 2% 64% 18
6 BCKDHA authKW 301440 1% 92% 12
7 BRANCHED CHAIN KETO ACIDS authKW 287438 1% 81% 13
8 BCKDHB authKW 244922 1% 100% 9
9 BRANCHED CHAIN ALPHA KETOACID DEHYDROGENASE KINASE authKW 217708 1% 100% 8
10 BRANCHED CHAIN AMINOTRANSFERASE authKW 170369 1% 52% 12

Web of Science journal categories


Rank Term Chi square Shr. of publ. in
class containing
term		 Class's shr. of
term's tot. occurrences		 #P with
term in
class
1 Biochemistry & Molecular Biology 2640 42% 0% 466
2 Nutrition & Dietetics 1234 9% 0% 104
3 Biophysics 788 11% 0% 118
4 Medicine, Research & Experimental 661 10% 0% 113
5 Endocrinology & Metabolism 647 10% 0% 116
6 Genetics & Heredity 503 10% 0% 113
7 Pediatrics 406 7% 0% 84
8 Biochemical Research Methods 350 7% 0% 76
9 Neurosciences 132 9% 0% 100
10 Chemistry, Analytical 116 6% 0% 66

Address terms


Rank Term Chi square Shr. of publ. in
class containing
term		 Class's shr. of
term's tot. occurrences		 #P with
term in
class
1 DR ZEINALIS MED GENET 81641 0% 100% 3
2 PROGRAMA POS CB BIOQUIM 62199 0% 57% 4
3 BIOL MOL SEVERO OCHOA CSIC UAMIDIPAZ 54427 0% 100% 2
4 ERROS INATOS METAB 36121 1% 15% 9
5 NENASC 32389 0% 24% 5
6 ALTHOUSE 31095 0% 29% 4
7 AMRITA NANO SCI MOL MED ACNSMM 27214 0% 100% 1
8 ANESTHESIOL MED PHYSIOLMOL BIOL 27214 0% 100% 1
9 ANESTHESIOL MOL MEDCARDIOVASC S 27214 0% 100% 1
10 ANESTHESIOLCARDIOVASC S MOL MED 27214 0% 100% 1

Journals


Rank Term Chi square Shr. of publ. in
class containing
term		 Class's shr. of
term's tot. occurrences		 #P with
term in
class
1 JOURNAL OF INHERITED METABOLIC DISEASE 11067 4% 1% 43
2 METABOLIC BRAIN DISEASE 4292 1% 1% 15
3 BIOCHEMICAL JOURNAL 3286 5% 0% 60
4 JOURNAL OF NUTRITION 3090 3% 0% 39
5 METHODS IN ENZYMOLOGY 2356 3% 0% 38
6 MOLECULAR GENETICS AND METABOLISM 2206 1% 1% 16
7 INTERNATIONAL JOURNAL OF BIOCHEMISTRY 1496 1% 0% 13
8 JOURNAL OF NUTRITIONAL SCIENCE AND VITAMINOLOGY 1475 1% 0% 12
9 ADVANCES IN ENZYME REGULATION 1132 0% 1% 5
10 ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS 1041 2% 0% 26

Author Key Words


Rank Term Chi square Shr. of publ. in
class containing
term		 Class's shr. of
term's tot. occurrences		 #P with
term in
class		 LCSH search Wikipedia search
1 MAPLE SYRUP URINE DISEASE 2816531 12% 75% 138 Search MAPLE+SYRUP+URINE+DISEASE Search MAPLE+SYRUP+URINE+DISEASE
2 BRANCHED CHAIN ALPHA KETO ACID DEHYDROGENASE COMPLEX 593422 2% 84% 26 Search BRANCHED+CHAIN+ALPHA+KETO+ACID+DEHYDROGENASE+COMPLEX Search BRANCHED+CHAIN+ALPHA+KETO+ACID+DEHYDROGENASE+COMPLEX
3 BRANCHED CHAIN ALPHA KETOACID DEHYDROGENASE 544901 3% 69% 29 Search BRANCHED+CHAIN+ALPHA+KETOACID+DEHYDROGENASE Search BRANCHED+CHAIN+ALPHA+KETOACID+DEHYDROGENASE
4 BRANCHED CHAIN AMINO ACIDS 361611 9% 13% 103 Search BRANCHED+CHAIN+AMINO+ACIDS Search BRANCHED+CHAIN+AMINO+ACIDS
5 MSUD 314887 2% 64% 18 Search MSUD Search MSUD
6 BCKDHA 301440 1% 92% 12 Search BCKDHA Search BCKDHA
7 BRANCHED CHAIN KETO ACIDS 287438 1% 81% 13 Search BRANCHED+CHAIN+KETO+ACIDS Search BRANCHED+CHAIN+KETO+ACIDS
8 BCKDHB 244922 1% 100% 9 Search BCKDHB Search BCKDHB
9 BRANCHED CHAIN ALPHA KETOACID DEHYDROGENASE KINASE 217708 1% 100% 8 Search BRANCHED+CHAIN+ALPHA+KETOACID+DEHYDROGENASE+KINASE Search BRANCHED+CHAIN+ALPHA+KETOACID+DEHYDROGENASE+KINASE
10 BRANCHED CHAIN AMINOTRANSFERASE 170369 1% 52% 12 Search BRANCHED+CHAIN+AMINOTRANSFERASE Search BRANCHED+CHAIN+AMINOTRANSFERASE

Core articles
The table includes core articles in the class. The following variables is taken into account for the relevance score of an article in a cluster c:
(1) Number of references referring to publications in the class.
(2) Share of total number of active references referring to publications in the class.
(3) Age of the article. New articles get higher score than old articles.
(4) Citation rate, normalized to year.


Rank Reference # ref.
in cl.		 Shr. of ref. in
cl.		 Citations
1 BURRAGE, LC , NAGAMANI, SCS , CAMPEAU, PM , LEE, BH , (2014) BRANCHED-CHAIN AMINO ACID METABOLISM: FROM RARE MENDELIAN DISEASES TO MORE COMMON DISORDERS.HUMAN MOLECULAR GENETICS. VOL. 23. ISSUE . P. R1 -R8 52 61% 13
2 SKVORAK, KJ , (2009) ANIMAL MODELS OF MAPLE SYRUP URINE DISEASE.JOURNAL OF INHERITED METABOLIC DISEASE. VOL. 32. ISSUE 2. P. 229 -246 48 53% 22
3 COUCE, ML , RAMOS, F , BUENO, MA , DIAZ, J , MEAVILLA, S , BOVEDA, MD , FERNANDEZ-MARMIESSE, A , GARCIA-CAZORLA, A , (2015) EVOLUTION OF MAPLE SYRUP URINE DISEASE IN PATIENTS DIAGNOSED BY NEWBORN SCREENING VERSUS LATE DIAGNOSIS.EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY. VOL. 19. ISSUE 6. P. 652 -659 26 90% 0
4 MITSUBUCHI, H , OWADA, M , ENDO, F , (2005) MARKERS ASSOCIATED WITH INBORN ERRORS OF METABOLISM OF BRANCHED-CHAIN AMINO ACIDS AND THEIR RELEVANCE TO UPPER LEVELS OF INTAKE IN HEALTHY PEOPLE: AN IMPLICATION FROM CLINICAL AND MOLECULAR INVESTIGATIONS ON MAPLE SYRUP URINGE DISEASE.JOURNAL OF NUTRITION. VOL. 135. ISSUE 6. P. 1565S-1570S 30 94% 17
5 HUTSON, SM , SWEATT, AJ , LANOUE, KF , (2005) BRACHED-CHAIN AMINO ACID METABOLISM: IMPLICATIONS FOR ESTABLISHING SAFE INTAKES'.JOURNAL OF NUTRITION. VOL. 135. ISSUE 6. P. 1557S-1564S 38 69% 50
6 FRAZIER, DM , ALLGEIER, C , HOMER, C , MARRIAGE, BJ , OGATA, B , ROHR, F , SPLETT, PL , STEMBRIDGE, A , SINGH, RH , (2014) NUTRITION MANAGEMENT GUIDELINE FOR MAPLE SYRUP URINE DISEASE: AN EVIDENCE- AND CONSENSUS-BASED APPROACH.MOLECULAR GENETICS AND METABOLISM. VOL. 112. ISSUE 3. P. 210-217 32 64% 10
7 LU, G , SUN, HP , SHE, PX , YOUN, JY , WARBURTON, S , PING, PP , VONDRISKA, TM , CAI, H , LYNCH, CJ , WANG, YB , (2009) PROTEIN PHOSPHATASE 2CM IS A CRITICAL REGULATOR OF BRANCHED-CHAIN AMINO ACID CATABOLISM IN MICE AND CULTURED CELLS.JOURNAL OF CLINICAL INVESTIGATION. VOL. 119. ISSUE 6. P. 1678-1687 26 76% 38
8 VILELA, TC , SCAINI, G , FURLANETTO, CB , PASQUALI, MAB , SANTOS, JPA , GELAIN, DP , MOREIRA, JCF , SCHUCK, PF , FERREIRA, GC , STRECK, EL , (2017) APOPTOTIC SIGNALING PATHWAYS INDUCED BY ACUTE ADMINISTRATION OF BRANCHED-CHAIN AMINO ACIDS IN AN ANIMAL MODEL OF MAPLE SYRUP URINE DISEASE.METABOLIC BRAIN DISEASE. VOL. 32. ISSUE 1. P. 115 -122 25 71% 0
9 FEIER, F , SCHWARTZ, IVD , BENKERT, AR , NETO, JS , MIURA, I , CHAPCHAP, P , DA FONSECA, EA , VIEIRA, S , ZANOTELLI, ML , VAIRO, FPE , ET AL (2016) LIVING RELATED VERSUS DECEASED DONOR LIVER TRANSPLANTATION FOR MAPLE SYRUP URINE DISEASE.MOLECULAR GENETICS AND METABOLISM. VOL. 117. ISSUE 3. P. 336 -343 22 71% 2
10 TSO, SC , QI, XB , GUI, WJ , CHUANG, JL , MORLOCK, LK , WALLACE, AL , AHMED, K , LAXMAN, S , CAMPEAU, PM , LEE, BH , ET AL (2013) STRUCTURE-BASED DESIGN AND MECHANISMS OF ALLOSTERIC INHIBITORS FOR MITOCHONDRIAL BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE KINASE.PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA. VOL. 110. ISSUE 24. P. 9728-9733 25 71% 12

Classes with closest relation at Level 1


Rank Class id link
1 4028 PYRUVATE DEHYDROGENASE COMPLEX//DIHYDROLIPOAMIDE DEHYDROGENASE//LIPOYL DOMAIN
2 14969 EXPT GENET//NMR METABOL//BIOINFORMAT SYST BIOL
3 28675 3 HYDROXYISOBUTYRATE DEHYDROGENASE//ECHS1//3 HYDROXYISOBUTYRIC ACID
4 27588 BETA HYDROXY BETA METHYLBUTYRATE//HMB//METAB TECHNOL INC
5 4465 INDICATOR AMINO ACID OXIDATION//AMINO ACID REQUIREMENTS//PROTEIN METABOLISM
6 25617 AMINO ACID IMBALANCE//FOOD INTAKE//ANTERIOR PIRIFORM CORTEX
7 20840 INFUSIONSTHERAPIE UND KLINISCHE ERNAHRUNG//HYPOCALORIC PARENTERAL NUTRITION//PROCONVULSANT DRUGS
8 20877 PHENYLALANINE DEHYDROGENASE//LEUCINE DEHYDROGENASE//VALINE DEHYDROGENASE
9 12928 METABOLIC ACIDOSIS//SERUM BICARBONATE//ACIDOSIS
10 3120 MUSCLE PROTEIN SYNTHESIS//EXERCISE METAB GRP//LEUCINE

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