Class information for:
Level 1: MARFAN SYNDROME//FIBRILLIN//FBN1

Basic class information
Class id #P Avg. number of
references		 Database coverage
of references
2309 2286 33.3 76%


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
420 3       ABDOMINAL AORTIC ANEURYSM//VASC SURG//JOURNAL OF VASCULAR SURGERY 28495
556 2             AORTIC DISSECTION//MARFAN SYNDROME//BICUSPID AORTIC VALVE 14567
2309 1                   MARFAN SYNDROME//FIBRILLIN//FBN1 2286

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 MARFAN SYNDROME authKW 5697813 29% 64% 669
2 FIBRILLIN authKW 1408562 7% 62% 170
3 FBN1 authKW 1089731 4% 88% 93
4 FIBRILLIN 1 authKW 1015259 5% 66% 116
5 LOEYS DIETZ SYNDROME authKW 994463 4% 85% 88
6 MICROFIBRILS authKW 406530 5% 29% 106
7 CONGENITAL CONTRACTURAL ARACHNODACTYLY authKW 287833 1% 86% 25
8 MARFAN authKW 267371 1% 65% 31
9 ECTOPIA LENTIS authKW 258311 2% 54% 36
10 FBN1 GENE authKW 256078 1% 91% 21

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 Cardiac & Cardiovascular System 6328 21% 0% 490
2 Genetics & Heredity 5541 21% 0% 487
3 Pediatrics 758 7% 0% 165
4 Peripheral Vascular Diseases 741 6% 0% 137
5 Ophthalmology 665 5% 0% 119
6 Anatomy & Morphology 604 2% 0% 55
7 Cell Biology 473 9% 0% 213
8 Respiratory System 462 4% 0% 98
9 Orthopedics 356 4% 0% 91
10 Biochemistry & Molecular Biology 314 14% 0% 325

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 MARFAN GRP 213692 1% 100% 16
2 REFERENCE SYNDROME MARFAN PARENTES 205464 1% 77% 20
3 CENT BIOCHIM HORMONOL GENET MOL 174513 1% 93% 14
4 REFERENCE SYNDROMES MARFAN PARENTES 120202 0% 100% 9
5 TRS OURCE RARE DISORDERS 91572 1% 57% 12
6 MUNROE HUMAN GENET 55647 0% 83% 5
7 SECT FUNCT STRUCTSAWARA KU 55647 0% 83% 5
8 U827 43680 1% 18% 18
9 BIOCHIM HORMONOL GENET MOL 40894 0% 44% 7
10 SONALEE 40067 0% 100% 3

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 AMERICAN JOURNAL OF MEDICAL GENETICS PART A 10304 3% 1% 67
2 MATRIX BIOLOGY 6677 1% 2% 26
3 CLINICAL GENETICS 5013 2% 1% 44
4 HUMAN MUTATION 4303 2% 1% 36
5 PROGRESS IN PEDIATRIC CARDIOLOGY 3012 0% 4% 6
6 JOURNAL OF MEDICAL GENETICS 1892 1% 1% 29
7 AMERICAN JOURNAL OF HUMAN GENETICS 1755 1% 0% 33
8 CONNECTIVE TISSUE RESEARCH 1668 1% 1% 15
9 PROGRESS IN MEDICAL GENETICS 1668 0% 13% 1
10 GENETICS IN MEDICINE 1598 1% 1% 14

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 MARFAN SYNDROME 5697813 29% 64% 669 Search MARFAN+SYNDROME Search MARFAN+SYNDROME
2 FIBRILLIN 1408562 7% 62% 170 Search FIBRILLIN Search FIBRILLIN
3 FBN1 1089731 4% 88% 93 Search FBN1 Search FBN1
4 FIBRILLIN 1 1015259 5% 66% 116 Search FIBRILLIN+1 Search FIBRILLIN+1
5 LOEYS DIETZ SYNDROME 994463 4% 85% 88 Search LOEYS+DIETZ+SYNDROME Search LOEYS+DIETZ+SYNDROME
6 MICROFIBRILS 406530 5% 29% 106 Search MICROFIBRILS Search MICROFIBRILS
7 CONGENITAL CONTRACTURAL ARACHNODACTYLY 287833 1% 86% 25 Search CONGENITAL+CONTRACTURAL+ARACHNODACTYLY Search CONGENITAL+CONTRACTURAL+ARACHNODACTYLY
8 MARFAN 267371 1% 65% 31 Search MARFAN Search MARFAN
9 ECTOPIA LENTIS 258311 2% 54% 36 Search ECTOPIA+LENTIS Search ECTOPIA+LENTIS
10 FBN1 GENE 256078 1% 91% 21 Search FBN1+GENE Search FBN1+GENE

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 ROBINSON, PN , ARTEAGA-SOLIS, E , BALDOCK, C , COLLOD-BEROUD, G , BOOMS, P , DE PAEPE, A , DIETZ, HC , GUO, G , HANDFORD, PA , JUDGE, DP , ET AL (2006) THE MOLECULAR GENETICS OF MARFAN SYNDROME AND RELATED DISORDERS.JOURNAL OF MEDICAL GENETICS. VOL. 43. ISSUE 10. P. 769-787 177 78% 163
2 FRANKEN, R , HEESTERBEEK, TJ , DE WAARD, V , ZWINDERMAN, AH , PALS, G , MULDER, BJM , GROENINK, M , (2014) DIAGNOSIS AND GENETICS OF MARFAN SYNDROME.EXPERT OPINION ON ORPHAN DRUGS. VOL. 2. ISSUE 10. P. 1049 -1062 125 98% 1
3 SAKAI, LY , KEENE, DR , RENARD, M , DE BACKER, J , (2016) FBN1: THE DISEASE-CAUSING GENE FOR MARFAN SYNDROME AND OTHER GENETIC DISORDERS.GENE. VOL. 591. ISSUE 1. P. 279 -291 97 87% 1
4 JENSEN, SA , HANDFORD, PA , (2016) NEW INSIGHTS INTO THE STRUCTURE, ASSEMBLY AND BIOLOGICAL ROLES OF 10-12 NM CONNECTIVE TISSUE MICROFIBRILS FROM FIBRILLIN-1 STUDIES.BIOCHEMICAL JOURNAL. VOL. 473. ISSUE . P. 827 -838 99 82% 0
5 VON KODOLITSCH, Y , DE BACKER, J , RENARD, M , CAMPENS, L , MOSQUERA, LM , DE PAEPE, A , COUCKE, P , CALLEWAERT, B , (2015) MARFAN SYNDROME AND RELATED HERITABLE THORACIC AORTIC ANEURYSMS AND DISSECTIONS.CURRENT PHARMACEUTICAL DESIGN. VOL. 21. ISSUE 28. P. 4061 -4075 104 78% 1
6 ZEYER, KA , REINHARDT, DP , (2015) ENGINEERED MUTATIONS IN FIBRILLIN-1 LEADING TO MARFAN SYNDROME ACT AT THE PROTEIN, CELLULAR AND ORGANISMAL LEVELS.MUTATION RESEARCH-REVIEWS IN MUTATION RESEARCH. VOL. 765. ISSUE . P. 7 -18 84 94% 1
7 COOK, JR , RAMIREZ, F , (2014) CLINICAL, DIAGNOSTIC, AND THERAPEUTIC ASPECTS OF THE MARFAN SYNDROME.PROGRESS IN HERITABLE SOFT CONNECTIVE TISSUE DISEASES. VOL. 802. ISSUE . P. 77 -94 96 81% 5
8 VERSTRAETEN, A , ALAERTS, M , VAN LAER, L , LOEYS, B , (2016) MARFAN SYNDROME AND RELATED DISORDERS: 25 YEARS OF GENE DISCOVERY.HUMAN MUTATION. VOL. 37. ISSUE 6. P. 524 -531 67 89% 4
9 ROBINSON, PN , GODFREY, M , (2000) THE MOLECULAR GENETICS OF MARFAN SYNDROME AND RELATED MICROFIBRILLOPATHIES.JOURNAL OF MEDICAL GENETICS. VOL. 37. ISSUE 1. P. 9 -25 129 69% 142
10 JENSEN, SA , ROBERTSON, IB , HANDFORD, PA , (2012) DISSECTING THE FIBRILLIN MICROFIBRIL: STRUCTURAL INSIGHTS INTO ORGANIZATION AND FUNCTION.STRUCTURE. VOL. 20. ISSUE 2. P. 215-225 79 85% 24

Classes with closest relation at Level 1


Rank Class id link
1 33110 MYHRE SYNDROME//GELEOPHYSIC DYSPLASIA//MICROSPHEROPHAKIA
2 9650 AORTIC VALVE REPAIR//AORTIC ROOT REPLACEMENT//AORTIC ROOT ANEURYSM
3 7972 BICUSPID AORTIC VALVE//QUADRICUSPID AORTIC VALVE//AORTOPATHY
4 19200 LTBP//ALPHA V BETA 6//INTEGRIN ALPHA V BETA 6
5 16395 CUTIS LAXA//FIBULIN 1//FIBULIN 5
6 7639 EHLERS DANLOS SYNDROME//JOINT HYPERMOBILITY//HYPERMOBILITY
7 26461 CIAPIN1//MG7 AG//ZNRD1
8 7939 DESMOSINE//RECH OPHTALMOL//ELASTIN PEPTIDES
9 18862 COLLAGEN VI//TYPE VI COLLAGEN//BETHLEM MYOPATHY
10 30234 MEGALOCORNEA//NEUHAUSER SYNDROME//ANTERIOR MEGALOPHTHALMOS

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