Class information for:
Level 1: 3 HYDROXY 3 METHYLGLUTARIC ACIDURIA//3 METHYLGLUTACONIC ACIDURIA//3 METHYLGLUTARIC ACID

Basic class information

Class id #P Avg. number of
references
Database coverage
of references
24942 296 23.3 67%



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
0 4 BIOCHEMISTRY & MOLECULAR BIOLOGY//CELL BIOLOGY//ONCOLOGY 4064930
389 3       PEROXISOME//CARNITINE//JOURNAL OF INHERITED METABOLIC DISEASE 30841
2144 2             JOURNAL OF INHERITED METABOLIC DISEASE//NEWBORN SCREENING//METHYLMALONIC ACIDEMIA 5035
24942 1                   3 HYDROXY 3 METHYLGLUTARIC ACIDURIA//3 METHYLGLUTACONIC ACIDURIA//3 METHYLGLUTARIC ACID 296

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 3 HYDROXY 3 METHYLGLUTARIC ACIDURIA authKW 1650553 5% 100% 16
2 3 METHYLGLUTACONIC ACIDURIA authKW 876839 6% 50% 17
3 3 METHYLGLUTARIC ACID authKW 561643 2% 78% 7
4 HMGCL authKW 515798 2% 100% 5
5 HMG COA LYASE authKW 464215 2% 75% 6
6 3 HYDROXY 3 METHYLGLUTARYL COA LYASE DEFICIENCY authKW 429830 2% 83% 5
7 HMG COA LYASE DEFICIENCY authKW 429830 2% 83% 5
8 3 METHYLGLUTACONIC ACIDURIA TYPE I authKW 412638 1% 100% 4
9 MEGDEL SYNDROME authKW 412638 1% 100% 4
10 3 HYDROXY 3 METHYLGLUTARYL COA LYASE authKW 412634 2% 67% 6

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 Genetics & Heredity 1796 33% 0% 97
2 Medicine, Research & Experimental 1348 26% 0% 76
3 Endocrinology & Metabolism 1056 24% 0% 70
4 Pediatrics 869 19% 0% 57
5 Medical Laboratory Technology 461 7% 0% 20
6 Clinical Neurology 338 16% 0% 47
7 Biochemistry & Molecular Biology 122 21% 0% 61
8 Neuroimaging 46 2% 0% 5
9 Neurosciences 14 7% 0% 20
10 Biophysics 11 3% 0% 10

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 GENET ENDOCRINE METAB DIS LGEM 368424 2% 71% 5
2 CLIN GENET FUNCT GENOM 232108 1% 75% 3
3 BAYLOR FDN 103160 0% 100% 1
4 BIOCHEM UNITUNIDAD BIOQUIM 103160 0% 100% 1
5 CHILDRENS HOSP WILHELMINA KINDERZIEKENHUIS 103160 0% 100% 1
6 CIENCIAS BASICAS DA SAUDE 103160 0% 100% 1
7 CLIN GENET FUNCT GEN 103160 0% 100% 1
8 CLIN GRP PEDIAT NEUROL 103160 0% 100% 1
9 DANA CHILDRENS HOSP MED 103160 0% 100% 1
10 GUYS ST THOMAS NHS FDN TRUSTS 103160 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 71589 19% 1% 56
2 MOLECULAR GENETICS AND METABOLISM 5575 4% 0% 13
3 EUROPEAN JOURNAL OF PEDIATRICS 2709 5% 0% 14
4 PEDIATRIC NEUROLOGY 1964 3% 0% 9
5 CLINICA CHIMICA ACTA 1567 4% 0% 13
6 ENZYME & PROTEIN 1144 0% 1% 1
7 JOURNAL OF NEUROGENETICS 760 1% 0% 2
8 NEUROGENETICS 679 1% 0% 2
9 AMERICAN JOURNAL OF HUMAN GENETICS 619 2% 0% 7
10 BRAIN & DEVELOPMENT 448 1% 0% 4

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 3 HYDROXY 3 METHYLGLUTARIC ACIDURIA 1650553 5% 100% 16 Search 3+HYDROXY+3+METHYLGLUTARIC+ACIDURIA Search 3+HYDROXY+3+METHYLGLUTARIC+ACIDURIA
2 3 METHYLGLUTACONIC ACIDURIA 876839 6% 50% 17 Search 3+METHYLGLUTACONIC+ACIDURIA Search 3+METHYLGLUTACONIC+ACIDURIA
3 3 METHYLGLUTARIC ACID 561643 2% 78% 7 Search 3+METHYLGLUTARIC+ACID Search 3+METHYLGLUTARIC+ACID
4 HMGCL 515798 2% 100% 5 Search HMGCL Search HMGCL
5 HMG COA LYASE 464215 2% 75% 6 Search HMG+COA+LYASE Search HMG+COA+LYASE
6 3 HYDROXY 3 METHYLGLUTARYL COA LYASE DEFICIENCY 429830 2% 83% 5 Search 3+HYDROXY+3+METHYLGLUTARYL+COA+LYASE+DEFICIENCY Search 3+HYDROXY+3+METHYLGLUTARYL+COA+LYASE+DEFICIENCY
7 HMG COA LYASE DEFICIENCY 429830 2% 83% 5 Search HMG+COA+LYASE+DEFICIENCY Search HMG+COA+LYASE+DEFICIENCY
8 3 METHYLGLUTACONIC ACIDURIA TYPE I 412638 1% 100% 4 Search 3+METHYLGLUTACONIC+ACIDURIA+TYPE+I Search 3+METHYLGLUTACONIC+ACIDURIA+TYPE+I
9 MEGDEL SYNDROME 412638 1% 100% 4 Search MEGDEL+SYNDROME Search MEGDEL+SYNDROME
10 3 HYDROXY 3 METHYLGLUTARYL COA LYASE 412634 2% 67% 6 Search 3+HYDROXY+3+METHYLGLUTARYL+COA+LYASE Search 3+HYDROXY+3+METHYLGLUTARYL+COA+LYASE

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 PIE, J , LOPEZ-VINAS, E , PUISAC, B , MENAO, S , PIE, A , CASALE, C , RAMOS, FJ , HEGARDT, FG , GOMEZ-PUERTAS, P , CASALS, N , (2007) MOLECULAR GENETICS OF HMG-COA LYASE DEFICIENCY.MOLECULAR GENETICS AND METABOLISM. VOL. 92. ISSUE 3. P. 198-209 54 84% 18
2 PIE, J , CASALS, N , PUISAC, B , HEGARDT, FG , (2003) MOLECULAR BASIS OF 3-HYDROXY-3-METHYLGLUTARIC ACIDURIA.JOURNAL OF PHYSIOLOGY AND BIOCHEMISTRY. VOL. 59. ISSUE 4. P. 311-321 32 82% 6
3 CARRASCO, P , MENAO, S , LOPEZ-VINAS, E , SANTPERE, G , CLOTET, J , SIERRA, AY , GRATACOS, E , PUISAC, B , GOMEZ-PUERTAS, P , HEGARDT, FG , ET AL (2007) C-TERMINAL END AND AMINOACID LYS(48) IN HMG-COA LYASE ARE INVOLVED IN SUBSTRATE BINDING AND ENZYME ACTIVITY.MOLECULAR GENETICS AND METABOLISM. VOL. 91. ISSUE 2. P. 120-127 23 92% 3
4 MENAO, S , LOPEZ-VINAS, E , MIR, C , PUISAC, B , GRATACOS, E , ARNEDO, M , CARRASCO, P , MORENO, S , RAMOS, M , GIL, MC , ET AL (2009) TEN NOVEL HMGCL MUTATIONS IN 24 PATIENTS OF DIFFERENT ORIGIN WITH 3-HYDROXY-3-METHYL-GLUTARIC ACIDURIA.HUMAN MUTATION. VOL. 30. ISSUE 3. P. E520 -E529 23 85% 1
5 WORTMANN, SB , KLUIJTMANS, LA , ENGELKE, UFH , WEVERS, RA , MORAVA, E , (2012) THE 3-METHYLGLUTACONIC ACIDURIAS: WHAT'S NEW?.JOURNAL OF INHERITED METABOLIC DISEASE. VOL. 35. ISSUE 1. P. 13 -22 33 49% 21
6 MUROI, J , YORIFUJI, T , UEMATSU, A , SHIGEMATSU, Y , ONIGATA, K , MARUYAMA, H , NOBUTOKI, T , KITAMURA, A , NAKAHATA, T , (2000) MOLECULAR AND CLINICAL ANALYSIS OF JAPANESE PATIENTS WITH 3-HYDROXY-3-METHYLGLUTARYL COA LYASE (HL) DEFICIENCY.HUMAN GENETICS. VOL. 107. ISSUE 4. P. 320 -326 23 88% 15
7 MONTGOMERY, C , MIZIORKO, HM , (2011) INFLUENCE OF MULTIPLE CYSTEINES ON HUMAN 3-HYDROXY-3-METHYLGLUTARYL-COA LYASE ACTIVITY AND FORMATION OF INTER-SUBUNIT ADDUCTS.ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS. VOL. 511. ISSUE 1-2. P. 48-55 16 94% 1
8 POSPISILOVA, E , MRAZOVA, L , HRDA, J , MARTINCOVA, O , ZEMAN, J , (2003) BIOCHEMICAL AND MOLECULAR ANALYSES IN THREE PATIENTS WITH 3-HYDROXY-3-METHYLGLUTARIC ACIDURIA.JOURNAL OF INHERITED METABOLIC DISEASE. VOL. 26. ISSUE 5. P. 433-441 20 91% 10
9 REIMAO, S , MORGADO, C , ALMEIDA, IT , SILVA, M , REAL, HC , CAMPOS, J , (2009) 3-HYDROXY-3-METHYLGLUTARYL-COENZYME A LYASE DEFICIENCY: INITIAL PRESENTATION IN A YOUNG ADULT.JOURNAL OF INHERITED METABOLIC DISEASE. VOL. 32. ISSUE 1. P. S49-S52 15 100% 6
10 CARDOSO, ML , RODRIGUES, MR , LEAO, E , MARTINS, E , DIOGO, L , RODRIGUES, E , GARCIA, P , ROLLAND, MO , VILARINHO, L , (2004) THE E37X IS A COMMON HMGCL MUTATION IN PORTUGUESE PATIENTS WITH 3-HYDROXY-3-METHYLGLUTARIC COA LYASE DEFICIENCY.MOLECULAR GENETICS AND METABOLISM. VOL. 82. ISSUE 4. P. 334-338 18 95% 15

Classes with closest relation at Level 1



Rank Class id link
1 10109 CARDIOLIPIN//BARTH SYNDROME//TAFAZZIN
2 36814 ALPHA KETOGLUTARIC ACID DEHYDROGENASE//ALPHA KETOGLUTARIC ACIDURIA//CHOROID PLEXUS CALCIFICATIONS
3 14230 BETA KETOTHIOLASE DEFICIENCY//MITOCHONDRIAL ACETOACETYL COA THIOLASE//THIOLASE
4 28675 3 HYDROXYISOBUTYRATE DEHYDROGENASE//ECHS1//3 HYDROXYISOBUTYRIC ACID
5 17020 GLUTARIC ACIDURIA TYPE I//GLUTARIC ACIDURIA TYPE 1//L 2 HYDROXYGLUTARIC ACIDURIA
6 20380 TERT BUTYLDIMETHYLSILYLATION//PROTEINOUS AMINO ACIDS//STAR SYMBOL PLOT
7 23687 ACETOACETYL COA SYNTHETASE//ACETOACETYL COA SYNTHETASE AACS//KETONE BODY UTILIZATION
8 9055 LEBERS HEREDITARY OPTIC NEUROPATHY//LHON//LEBER HEREDITARY OPTIC NEUROPATHY
9 2388 NEWBORN SCREENING//MEDIUM CHAIN ACYL COA DEHYDROGENASE DEFICIENCY//UNIT MOL MED
10 9072 METHYLMALONIC ACIDEMIA//PROPIONIC ACIDEMIA//METHYLMALONIC ACIDURIA

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