Description
This panel contains genes associated with osteogenesis imperfecta and osteoporosis. 

It has been compared against the Genomics England PanelApp 'Osteogenesis imperfecta' panel V5.1, with all discrepancies reviewed and resolved (November 2025).

12 reviewers

  • Paul De Fazio (Victorian Clinical Genetics Services)

  • Ain Roesley (Victorian Clinical Genetics Services)

  • Chirag Patel (Genetic Health Queensland)

  • Sangavi Sivagnanasundram (Melbourne Health)

  • Paul De Fazio (Victorian Clinical Genetics Services)

  • Bryony Thompson (Royal Melbourne Hospital)

  • Seb Lunke (Victorian Clinical Genetics Services)

  • Dean Phelan (Victorian Clinical Genetics Services)

  • Chern Lim (Victorian Clinical Genetics Services)

  • Tashunka Taylor-Miller (Victorian Clinical Genetics Services)

  • Zornitza Stark (Victorian Clinical Genetics Services)

  • Krithika Murali (Victorian Clinical Genetics Services)

47 Entities

47 reviewed, 43 green

List Entity Reviews Mode of inheritance Details
47 Entitiess
Green Green List (high evidence)
ALPL
1 review
1 green
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Hypophosphatasia, adult 146300 (AD, AR)
  • Hypophosphatasia, childhood 241510 AR
  • Hypophosphatasia, infantile 241500 AR
  • Odontohypophosphatasia 146300 AD, AR
Tags
  • treatable
Green Green List (high evidence)
ANO5
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Gnathodiaphyseal dysplasia MIM#166260
Tags
Green Green List (high evidence)
B3GALT6
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Spondyloepimetaphyseal dysplasia with joint laxity, type 1, with or without fractures, MIM# 271640, MONDO:0010075
Tags
Green Green List (high evidence)
B4GALT7
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Ehlers-Danlos syndrome, spondylodysplastic type, 1, MIM# 130070
Tags
Green Green List (high evidence)
BMP1
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type XIII , MIM#614856
Tags
Green Green List (high evidence)
CASR
1 review
1 green
BOTH monoallelic and biallelic (but BIALLELIC mutations cause a more SEVERE disease form), autosomal or pseudoautosomal
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Hyperparathyroidism, neonatal, MIM# 239200
  • severe hypercalcemia, bone demineralization, multiple fractures
Tags
Green Green List (high evidence)
CCDC134
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Osteogenesis imperfecta, type XXII, MIM#619795
Tags
Green Green List (high evidence)
COL1A1
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • combined osteogenesis imperfecta and Ehlers-Danlos syndrome 1 MONDO:0030854
  • Osteogenesis imperfecta type 1 MONDO:0008146
  • Osteogenesis imperfecta type 2 MONDO:0008147
  • Osteogenesis imperfecta type 3 MONDO:0009804
  • Osteogenesis imperfecta type 4
  • MONDO:0008148
Tags
Green Green List (high evidence)
COL1A2
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta type 1 MONDO:0008146
  • Osteogenesis imperfecta type 2 MONDO:0008147
  • Osteogenesis imperfecta type 3 MONDO:0009804
  • Osteogenesis imperfecta type 4 MONDO:0008148
Tags
Green Green List (high evidence)
COPB2
1 review
1 green
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Literature
Phenotypes
  • Osteoporosis, childhood- or juvenile-onset, with developmental delay, MIM# 619884
Tags
Green Green List (high evidence)
CREB3L1
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type XVI, 616229
Tags
Green Green List (high evidence)
CRTAP
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type VII MIM#610682
Tags
Green Green List (high evidence)
EMILIN1
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Literature
Phenotypes
  • arterial tortuosity-bone fragility syndrome MONDO:0971179
Tags
Green Green List (high evidence)
FAM46A
1 review
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type XVIII, MIM# 617952
Tags
  • new gene name
Green Green List (high evidence)
FKBP10
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • osteogenesis imperfecta type 11 MONDO:0012592
  • Bruck syndrome MONDO:0017195
Tags
Green Green List (high evidence)
GORAB
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Geroderma osteodysplasticum, MIM# 231070
Tags
Green Green List (high evidence)
IFITM5
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta type V, MIM#610967
Tags
Green Green List (high evidence)
KDELR2
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review
  • Expert Review Green
Phenotypes
  • Osteogenesis imperfecta 21, MIM# 619131
  • Increased susceptibility to fractures
  • joint hypermobility
  • Scoliosis
  • Bowing of the legs
  • Bowing of the arms
Tags
Green Green List (high evidence)
KIF5B
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert Review Green
  • Literature
Phenotypes
  • osteogenesis imperfecta, MONDO:0019019
Tags
Green Green List (high evidence)
LRP5
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • osteoporosis-pseudoglioma syndrome MONDO:0009820
Tags
Green Green List (high evidence)
MESD
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Other
Phenotypes
  • Osteogenesis imperfecta, type XX, MIM# 618644
Tags
Green Green List (high evidence)
NBAS
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • NHS GMS
Phenotypes
  • short stature
  • bone fragility
  • developmental delay
  • immunodeficiency
  • autism
  • Short stature, optic nerve atrophy, and Pelger-Huet anomaly, MIM# 614800
Tags
Green Green List (high evidence)
NOTCH2
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Hajdu-Cheney syndrome (MIM#102500)
Tags
Green Green List (high evidence)
P3H1
2 reviews
2 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type VIII, (MIM# 610915)
Tags
  • founder
Green Green List (high evidence)
P4HB
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Cole-Carpenter syndrome 1, MIM#112240
Tags
Green Green List (high evidence)
PLOD2
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Bruck syndrome 2, MIM# 609220
Tags
Green Green List (high evidence)
PLS3
1 review
1 green
X-LINKED: hemizygous mutation in males, monoallelic mutations in females may cause disease (may be less severe, later onset than males)
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • X-linked osteoporosis with fractures MONDO:0018315
Tags
Green Green List (high evidence)
PPIB
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type IX, MIM# 259440
Tags
Green Green List (high evidence)
SEC24D
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Cole-Carpenter syndrome 2, MIM# 616294
Tags
Green Green List (high evidence)
SERPINF1
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type VI, MIM# 613982
Tags
Green Green List (high evidence)
SERPINH1
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type X, MIM# 613848
Tags
Green Green List (high evidence)
SGMS2
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Calvarial doughnut lesions with bone fragility with or without spondylometaphyseal dysplasia MIM#126550
Tags
Green Green List (high evidence)
SP7
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta type 12, MONDO:0013460
  • Osteogenesis imperfecta, type XII, OMIM:613849
Tags
Green Green List (high evidence)
SPARC
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type XVII, MIM# 616507
Tags
Green Green List (high evidence)
SUCO
2 reviews
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Syndromic disease (MONDO:0002254), SUCO-related
Tags
Green Green List (high evidence)
TAPT1
3 reviews
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteochondrodysplasia, complex lethal, Symoens-Barnes-Gistelinck type (MIM#616897)
Tags
Green Green List (high evidence)
TMEM38B
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type XIV , MIM#615066
Tags
Green Green List (high evidence)
TNFRSF11B
1 review
1 green
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Literature
Phenotypes
  • juvenile Paget disease MONDO:0009394
  • chondrocalcinosis 1 MONDO:0010917
Tags
Green Green List (high evidence)
TRPV6
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Hyperparathyroidism, transient neonatal, MIM# 618188
Tags
Green Green List (high evidence)
UNC45A
3 reviews
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteootohepatoenteric syndrome, MIM# 619377
  • cholestasis
  • congenital diarrhea
  • impaired hearing
  • bone fragility
Tags
Green Green List (high evidence)
WNT1
2 reviews
2 green
BOTH monoallelic and biallelic (but BIALLELIC mutations cause a more SEVERE disease form), autosomal or pseudoautosomal
Sources
  • Expert Review Green
  • Victorian Clinical Genetics Services
Phenotypes
  • Osteogenesis imperfecta, type XV, MIM# 615220
  • Osteoporosis MONDO:0005298
Tags
Green Green List (high evidence)
WNT11
1 review
1 green
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Sources
  • Expert Review Green
  • Literature
Phenotypes
  • Osteoporosis, MONDO:0005298, WNT11-related
Tags
Green Green List (high evidence)
XYLT2
1 review
1 green
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert list
  • Expert Review Green
Phenotypes
  • Spondyloocular syndrome MIM#605822
Tags
Amber Amber List (moderate evidence)
MBTPS2
1 review
X-LINKED: hemizygous mutation in males, biallelic mutations in females
Sources
  • Expert list
  • Expert Review Amber
Phenotypes
  • Osteogenesis imperfecta, type XIX, MIM# 301014
Tags
Amber Amber List (moderate evidence)
PHLDB1
2 reviews
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert Review Amber
  • Literature
Phenotypes
  • Osteogenesis imperfecta, type XXIII, MIM# 620639
Tags
Red Red List (low evidence)
NUDT6
1 review
1 red
BIALLELIC, autosomal or pseudoautosomal
Sources
  • Expert List
  • Expert Review Red
Phenotypes
  • Osteogenesis imperfecta, MONDO:0019019, NUDT6-related
Tags
Red Red List (low evidence)
WNT4
1 review
1 red
Unknown
Sources
  • Expert list
Phenotypes
  • Osteoporosis
Tags

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