Class information for:
Level 1: GM2 GANGLIOSIDOSIS//SANDHOFF DISEASE//TAY SACHS DISEASE

Basic class information
ID Publications Average number
of references		 Avg. shr. active
ref. in WoS
10710 974 30.0 62%


Bar chart of Publication_year

Last years might be incomplete

Classes in level above (level 2)


ID, lev.
above		 Publications Label for level above
330 17366 FABRY DISEASE//GAUCHER DISEASE//MUCOPOLYSACCHARIDOSES

Terms with highest relevance score


Rank Term Type of term Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ. in
class
1 GM2 GANGLIOSIDOSIS Author keyword 114 82% 7% 67
2 SANDHOFF DISEASE Author keyword 113 77% 8% 77
3 TAY SACHS DISEASE Author keyword 66 58% 8% 76
4 HEXOSAMINIDASE Author keyword 23 44% 4% 41
5 HEXA GENE Author keyword 20 100% 1% 9
6 HEXOSAMINIDASE A Author keyword 19 74% 1% 14
7 HEXB Author keyword 18 83% 1% 10
8 GM2 GANGLIOSIDE Author keyword 15 48% 2% 23
9 BETA HEXOSAMINIDASE A Author keyword 12 75% 1% 9
10 N ACETYL BETA HEXOSAMINIDASE Author keyword 12 86% 1% 6

Web of Science journal categories

Author Key Words


Rank Web of Science journal category Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ. in
class		 LCSH search Wikipedia search
1 GM2 GANGLIOSIDOSIS 114 82% 7% 67 Search GM2+GANGLIOSIDOSIS Search GM2+GANGLIOSIDOSIS
2 SANDHOFF DISEASE 113 77% 8% 77 Search SANDHOFF+DISEASE Search SANDHOFF+DISEASE
3 TAY SACHS DISEASE 66 58% 8% 76 Search TAY+SACHS+DISEASE Search TAY+SACHS+DISEASE
4 HEXOSAMINIDASE 23 44% 4% 41 Search HEXOSAMINIDASE Search HEXOSAMINIDASE
5 HEXA GENE 20 100% 1% 9 Search HEXA+GENE Search HEXA+GENE
6 HEXOSAMINIDASE A 19 74% 1% 14 Search HEXOSAMINIDASE+A Search HEXOSAMINIDASE+A
7 HEXB 18 83% 1% 10 Search HEXB Search HEXB
8 GM2 GANGLIOSIDE 15 48% 2% 23 Search GM2+GANGLIOSIDE Search GM2+GANGLIOSIDE
9 BETA HEXOSAMINIDASE A 12 75% 1% 9 Search BETA+HEXOSAMINIDASE+A Search BETA+HEXOSAMINIDASE+A
10 N ACETYL BETA HEXOSAMINIDASE 12 86% 1% 6 Search N+ACETYL+BETA+HEXOSAMINIDASE Search N+ACETYL+BETA+HEXOSAMINIDASE

Key Words Plus


Rank Web of Science journal category Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ. in
class
1 GM2 GANGLIOSIDOSIS 134 71% 11% 107
2 TAY SACHS DISEASE 109 42% 20% 197
3 GM2 GANGLIOSIDOSIS B1 VARIANT 94 94% 3% 33
4 ADULT GM2 GANGLIOSIDOSIS 83 94% 3% 30
5 SANDHOFF DISEASE 60 52% 8% 81
6 HUMAN BETA HEXOSAMINIDASE 44 70% 4% 37
7 HEXOSAMINIDASE A DEFICIENCY 40 82% 2% 23
8 BETA HEXOSAMINIDASE 39 35% 9% 89
9 HEXA GENE 36 69% 3% 31
10 ASHKENAZI JEWISH 36 77% 2% 24

Journals

Reviews


Title Publ. year Cit. Active references % act. ref.
to same field
Biochemical consequences of mutations causing the GM2 gangliosidoses 1999 128 195 71%
The natural history of juvenile or subacute GM2 gangliosidosis: 21 new cases and literature review of 134 previously reported 2006 32 71 94%
Naturally occurring mutations in G(M2) gangliosidosis: A compendium 2001 15 119 92%
Tay-Sachs disease carrier screening: A model for prevention of genetic disease 1998 22 132 86%
Juvenile Sandhoff disease - Nine new cases and a review of the literature 2004 12 4 100%
Tay-Sachs disease: From clinical description to molecular defect 2001 7 12 100%
THE BIOCHEMISTRY OF HEXA AND HEXB GENE-MUTATIONS CAUSING GM2 GANGLIOSIDOSIS 1991 36 63 81%
Miglustat as a therapeutic agent: prospects and caveats 2012 9 60 23%
NATURAL-HISTORY AND INHERITED DISORDERS OF A LYSOSOMAL-ENZYME, BETA-HEXOSAMINIDASE 1989 120 43 77%
Molecular pathogenesis and therapeutic targets of lysosomal diseases 2007 0 12 100%

Address terms


Rank Address term Relevance score
(tfidf)		 Class's shr.
of term's tot.
occurrences		 Shr. of publ.
in class containing
term		 Num. of
publ.
in class
1 GEN SURG ENDOCRINOL 1 11 78% 0.7% 7
2 BIOCHIM MED MALAD METAB 2 67% 0.2% 2
3 ZAKLAD MED RATUNKOWEJ KATASTROF 2 67% 0.2% 2
4 MED BIOL SCI DSMB 2 50% 0.3% 3
5 ESTHET MED 1 38% 0.3% 3
6 EMERGENCY MED DISASTERS 1 24% 0.5% 5
7 NEUROLNEUROMUSCULAR 1 33% 0.2% 2
8 PEDIAT ORTHOPED TRAUMATOL 1 13% 0.5% 5
9 ADV THER IES PROGRAM 1 50% 0.1% 1
10 CATEDRA PEDIAT NEONATOL 1 50% 0.1% 1

Related classes at same level (level 1)


Rank Relatedness score Related classes
1 0.0000235834 PROSAPOSIN//SPHINGOLIPID ACTIVATOR PROTEIN//SAPOSIN B
2 0.0000123930 GALACTOSIALIDOSIS//GM1 GANGLIOSIDOSIS//SIALIDASE
3 0.0000122251 N ACETYL BETA D GLUCOSAMINIDASE//URINARY ENZYMES//RATE ASSAY
4 0.0000101152 FARBER DISEASE//ASAH1//FARBER LIPOGRANULOMATOSIS
5 0.0000101042 FUCOSIDOSIS//ALPHA L FUCOSIDASE//FUCOSE DEHYDROGENASE
6 0.0000092590 METACHROMATIC LEUKODYSTROPHY//ARYLSULFATASE A//MULTIPLE SULFATASE DEFICIENCY
7 0.0000074816 GLOBOID CELL LEUKODYSTROPHY//KRABBE DISEASE//PSYCHOSINE
8 0.0000070551 ACROSOMAL ENZYME//DEXAMETHSONE//SPERM CHARACTERISTIC
9 0.0000070480 GAUCHER DISEASE//GLUCOCEREBROSIDASE//GAUCHERS DISEASE
10 0.0000070053 I CELL DISEASE//MANNOSE 6 PHOSPHATE//MUCOLIPIDOSIS TYPE III