Genes associated with “muscular dystrophy

86 genes foundHPO: Muscular dystrophyOpen Targets: muscular dystrophy26089 ClinVar P/LP variants3 PanelApp panels
How are genes scored? (0–100 composite)
-5–15
ClinGen
0–15
HPO Freq
0–15
Open Targets
0–12
Phen2Gene
0–10
ClinVar
0–8
Constraint
0–8
Dosage
0–8
OMIM CS
0–8
PanelApp
0–5
OMIM
0–5
G2P
0–4
Breadth
Tiers:Strong Candidates (≥20)Consider (≥8)Possible (≥3)
Evidence dots:
HPOClinVarPhen2GeneOpen TargetsPanelApp
hover for details

Strong Candidates

43 genes
1
DMD

dystrophin

63
score
ClinGen: DefinitivePanel: Green (2)P2G #25GTR ↑

Duchenne muscular dystrophy

Frequency
-
P/LP Variants
90
OT Score
0.89
2
LARGE1

LARGE xylosyl- and glucuronyltransferase 1

56
score
ClinGen: DefinitivePanel: GreenGTR ↑

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A6

Frequency
-
P/LP Variants
97
OT Score
0.86
3
DYSF

dysferlin

51
score
ClinGen: DefinitivePanel: AmberP2G #1GTR ↑

Miyoshi muscular dystrophy 1

Frequency
-
P/LP Variants
1
OT Score
0.88
4
LAMA2

laminin subunit alpha 2

50
score
ClinGen: DefinitivePanel: GreenP2G #9GTR ↑

congenital merosin-deficient muscular dystrophy 1A

Frequency
-
P/LP Variants
3
OT Score
0.85
5
LMNA

lamin A/C

49
score
ClinGen: DefinitiveACMG SFPanel: GreenP2G #10GTR ↑

congenital muscular dystrophy due to LMNA mutation

Frequency
100%
n=15
P/LP Variants
283
OT Score
0.88
6
B3GALNT2

beta-1,3-N-acetylgalactosaminyltransferase 2

48
score
ClinGen: DefinitivePanel: GreenP2G #35GTR ↑

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 11

Frequency
-
P/LP Variants
2
OT Score
0.84
7
TRAPPC11

trafficking protein particle complex subunit 11

47
score
ClinGen: DefinitivePanel: GreenGTR ↑

autosomal recessive limb-girdle muscular dystrophy type R18

Frequency
100%
n=8
P/LP Variants
1
OT Score
0.82
8
JAG2

jagged canonical Notch ligand 2

45
score
ClinGen: ModeratePanel: GreenGTR ↑

muscular dystrophy, limb-girdle, autosomal recessive 27

Frequency
86%
n=14
P/LP Variants
14
OT Score
0.79
9
PLEC

plectin

45
score
ClinGen: DefinitivePanel: GreenP2G #48GTR ↑

autosomal recessive limb-girdle muscular dystrophy type 2Q

Frequency
-
P/LP Variants
2
OT Score
0.83
44POMT1
DefP:G#6

muscular dystrophy-dystroglycanopathy (congenital with intellectual disability), type B1

41FKTN
DefP:G#5

autosomal recessive limb-girdle muscular dystrophy type 2M

41ANO5
DefP:R#2

Miyoshi muscular dystrophy 3

39POMGNT1
DefP:G#14

muscular dystrophy-dystroglycanopathy (congenital with intellectual disability), type B3

39POMT2
DefP:G#7

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A2

38SMCHD1
DefP:G×2

facioscapulohumeral muscular dystrophy 2

37DAG1
DefP:G

autosomal recessive limb-girdle muscular dystrophy type 2P

37COL12A1
P:G

Ullrich congenital muscular dystrophy 2

37GMPPB
DefP:G

muscular dystrophy-dystroglycanopathy (congenital with intellectual disability), type B14

37DPM3
ModP:G

DPM3-congenital disorder of glycosylation

36SYNE1
DefP:G

Emery-Dreifuss muscular dystrophy 4, autosomal dominant

36COL6A1
DefP:G#11

Ullrich congenital muscular dystrophy 1A

35CAPN3
Def#19

autosomal recessive limb-girdle muscular dystrophy type 2A

35POMGNT2
DefP:G#17

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 8

35TCAP
DefP:A

autosomal recessive limb-girdle muscular dystrophy type 2G

34SGCG
Def

autosomal recessive limb-girdle muscular dystrophy type 2C

34FKRP
DefP:G#16

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A5

34TRIM32
Def

autosomal recessive limb-girdle muscular dystrophy type 2H

32CRPPA
Def

autosomal recessive limb-girdle muscular dystrophy type 2U

32SGCA
Def

autosomal recessive limb-girdle muscular dystrophy type 2D

32SGCB
Def

autosomal recessive limb-girdle muscular dystrophy type 2E

31SGCD
Def

autosomal recessive limb-girdle muscular dystrophy type 2F

31TTN
DefSF

tibial muscular dystrophy

31COL6A3
DefP:G#12

collagen type VI alpha 3 chain

31DNAJB6
Def

autosomal dominant limb-girdle muscular dystrophy type 1D (DNAJB6)

28B4GAT1
ModP:G

muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A13

26COL6A2
DefP:G#15

collagen type VI alpha 2 chain

25SELENON
DefP:G

rigid spine muscular dystrophy 1

24POGLUT1
DefP:G

autosomal recessive limb-girdle muscular dystrophy type 2R1

24DPM2
ModP:G

dolichyl-phosphate mannosyltransferase subunit 2, regulatory

autosomal recessive limb-girdle muscular dystrophy type 2Y

21FHL1
P:G

four and a half LIM domains 1

20HNRNPDL
Def#20

autosomal dominant limb-girdle muscular dystrophy type 1G

20TNPO3
Def

autosomal dominant limb-girdle muscular dystrophy type 1F

Consider

21 genes
19MYH7
DefSF

myosin heavy chain 7

18NR0B1
#3

X-linked adrenal hypoplasia congenita

17RXYLT1
Def

ribitol xylosyltransferase 1

16FLNC
DefSF

filamin C

fatal infantile hypertonic myofibrillar myopathy

15TUBB3
Def

tubulin beta 3 class III

14LDB3
Str

LIM domain binding 3

EPIDERMOLYSIS BULLOSA SIMPLEX 5B, WITH MUSCULAR DYSTROPHY; EBS5B

MUSCULAR DYSTROPHY, LIMB-GIRDLE, AUTOSOMAL RECESSIVE 17; LGMDR17

MUSCULAR DYSTROPHY, CONGENITAL, MEGACONIAL TYPE; MDCMC

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (CONGENITAL WITH BRAIN AND EYE ANOMALIES), TYPE A, 1; MDDGA1

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (CONGENITAL WITH BRAIN AND EYE ANOMALIES), TYPE A, 3; MDDGA3

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (CONGENITAL WITH BRAIN AND EYE ANOMALIES), TYPE A, 4; MDDGA4

MIYOSHI MUSCULAR DYSTROPHY 1; MMD1

12DST
Def
12CAVIN1
DefP:G

congenital generalized lipodystrophy type 4

10RYR1
DefSFP:R#4

congenital multicore myopathy with external ophthalmoplegia

10DPM1
DefP:A

congenital disorder of glycosylation type 1E

10ITGA7
P:G#13

congenital muscular dystrophy due to integrin alpha-7 deficiency

congenital muscular dystrophy-respiratory failure-skin abnormalities-joint hyperlaxity syndrome

8TMEM43
DefSFP:R

Emery-Dreifuss muscular dystrophy 7, autosomal dominant

Possible

17 genes — click to expand
6CAV3
Def#18

creatine phosphokinase, elevated serum

6BET1
P:G

muscular dystrophy, congenital, with rapid progression

6LIPE
Def

LIPE-related familial partial lipodystrophy

6BAG3
DefSF

myofibrillar myopathy 6

5SYNE2
P:R

Emery-Dreifuss muscular dystrophy 5, autosomal dominant

EMERY-DREIFUSS MUSCULAR DYSTROPHY 2, AUTOSOMAL DOMINANT; EDMD2

Facioscapulohumeral muscular dystrophy 1

MUSCULAR DYSTROPHY, LIMB-GIRDLE, AUTOSOMAL DOMINANT 1; LGMDD1

MUSCULAR DYSTROPHY, LIMB-GIRDLE, AUTOSOMAL RECESSIVE 1; LGMDR1

MUSCULAR DYSTROPHY, LIMB-GIRDLE, AUTOSOMAL RECESSIVE 5; LGMDR5

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (LIMB-GIRDLE), TYPE C, 14; MDDGC14

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (LIMB-GIRDLE), TYPE C, 2; MDDGC2

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (LIMB-GIRDLE), TYPE C, 3; MDDGC3

MUSCULAR DYSTROPHY-DYSTROGLYCANOPATHY (LIMB-GIRDLE), TYPE C, 9; MDDGC9

ULLRICH CONGENITAL MUSCULAR DYSTROPHY 1A; UCMD1A

Data aggregated from HPO/Monarch, ClinVar, OMIM (phenotypes + clinical synopsis), Open Targets, PanelApp, Phen2Gene, and Gene2Phenotype. Click a gene to view full details.