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Phenotypes for disease #02219 (CMTX1 (Charcot-Marie-Tooth disease, X-linked dominant, type 1 (CMTX1)), OMIM:302800)
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Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Phenotype details
: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial
: initial diagnosis, before molecular testing
Diagnosis/Definite
: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance
: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
Unknown
Familial
Familial, autosomal dominant
Familial, autosomal recessive
Familial, X-linked
Familial, X-linked dominant
Familial, X-linked dominant, male sparing
Familial, X-linked recessive
Paternal, Y-linked
Maternal, mitochondrial
Isolated (sporadic)
Di-genic
Complex
- = Not applicable
Age/Examination
: age at which the individual was examined.
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Diagnosis
: age diagnosis was confirmed
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Onset
: Age first symptoms disease appeared in individual:
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Phenotype/Onset
: individual's phenotype at Age/Onset described using HPO
Protein
: result from protein staining
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Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
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|
Text
Arg|Ser
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!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
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=""
Text
="p.0"
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!=""
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!=""
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!=""
Text
!="p.0"
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combination
Text
*|Ter !fs
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Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
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Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
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19 entries on 1 page. Showing entries 1 - 19.
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How to query
Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000242301
clinical suspicion of HS(A)N, Charcot foot, sensitive axonal PNP, wound healing disorder
-
-
Unknown
-
60y
-
-
-
Andreas Laner
00320260
0000291696
see paper; Gait disturbance (HP:0001288), Pes cavus (HP:0001761), Upper limb muscle weakness (HP:0003484), Upper limb amyotrophy (HP:0009129), Lower limb amyotrophy (HP:0007210), Reduced tendon reflexes (HP:0001315), Distal sensory impairment (HP:0002936)
Charcot-Marie-Tooth disease
CMTX1
Familial, X-linked recessive
49y
-
10y-20y
Lower limb muscle weakness (HP:0007340)
-
Farina Kemper
00398575
0000291697
see paper; Gait disturbance (HP:0001288), Pes cavus (HP:0001761), Upper limb muscle weakness (HP:0003484), Upper limb amyotrophy (HP:0009129), Lower limb amyotrophy (HP:0007210), Reduced tendon reflexes (HP:0001315)
Charcot-Marie-Tooth disease
CMTX1
Familial, X-linked recessive
35y
-
>10y
Lower limb muscle weakness (HP:0007340)
-
Farina Kemper
00398593
0000291698
see paper; Gait disturbance (HP:0001288), Pes cavus (HP:0001761), Upper limb muscle weakness (HP:0003484), Upper limb amyotrophy (HP:0009129), Lower limb amyotrophy (HP:0007210), Reduced tendon reflexes (HP:0001315), Hand tremor (HP:0002378), No pyramidal signs (-HP:0002936), EMG abnormality (HP:0003457)
Charcot-Marie-Tooth disease
CMTX1
Familial, X-linked recessive
25y
-
14y
Lower limb muscle weakness (HP:0007340)
-
Farina Kemper
00398594
0000291699
see paper; No pyramidal signs (-HP:0001288), No pyramidal signs (-HP:0001761), No pyramidal signs (-HP:0003484), No pyramidal signs (-HP:0007340), Upper limb amyotrophy (HP:0009129), No pyramidal signs (-HP:0001315), No pyramidal signs (-HP:0002378), No pyramidal signs (-HP:0002936), EMG abnormality (HP:0003457)
Charcot-Marie-Tooth disease
CMTX1
Familial, X-linked recessive
25y
-
>20y
Lower limb amyotrophy (HP:0007210)
-
Farina Kemper
00398595
0000292068
see paper;…, symmetrical muscle atrophy distal amyotrophy (HP:0003693); foot dorsiflexor weakness (HP:0009027); pes cavus (HP:0001761); reduced tendon reflexes (HP:0001315); mixed demyelinating and axonal polyneuropathy (HP:0007327)
CMT1X
-
Familial, X-linked dominant
32y
-
20y
distal lower limb muscle weakness (HP:0009053)
-
Maeve Soen
00398979
0000292578
see paper;…, symmetrical muscle atrophy distal amyotrophy (HP:0003693); distal lower limb muscle weakness (HP:0009053); pes cavus (HP:0001761); reduced tendon reflexes (HP:0001315)
CMT1X
-
Familial, X-linked dominant
57y
-
50y
-
-
Maeve Soen
00399473
0000292579
see paper;…, symmetrical muscle atrophy distal amyotrophy (HP:0003693); distal lower limb muscle weakness (HP:0009053); pes cavus (HP:0001761); reduced tendon reflexes (HP:0001315); gait disturbance (HP:0001288)
CMT1X
-
Familial, X-linked dominant
60y
-
30y
-
-
Maeve Soen
00399474
0000292580
see paper;…, symmetrical muscle atrophy distal amyotrophy (HP:0003693); distal lower limb muscle weakness (HP:0009053); pes cavus (HP:0001761); reduced tendon reflexes (HP:0001315)
CMT1X
-
Familial, X-linked dominant
55y
-
30y
-
-
Maeve Soen
00399475
0000292581
see paper;…, distal lower limb muscle weakness (HP:0009053); steppage gait (HP:0003376); pes cavus (HP:0001761); reduced tendon reflexes (HP:0001315); peripheral neuropathy (HP:0009830); mixed demyelinating and axonal polyneuropathy (HP:0007327)
CMT1X
-
Familial, X-linked dominant
11y
-
06y
gait disturbance (HP:0001288)
-
Maeve Soen
00399476
0000292582
see paper;…, decreased compound muscle action potential amplitude (HP:0033383)
-
CMT1X
Familial, X-linked dominant
41y
41y
-
-
-
Maeve Soen
00399477
0000292669
see paper; ..., steppage gait (HP:0003376); pes cavus (HP:0001761); reduced tendon reflexes (HP:0001315); distal amyotrophy (HP:0003693); areflexia (HP:0001284); clubbing of fingers (HP:0100759); soft tissue swelling of interphalangeal joints (HP:0006162); mixed demyelinating and axonal polyneuropathy (HP:0007327); decreased compound muscle action potential amplitude (HP:0033383)
CMT1X
-
Familial, X-linked
20y
-
16y
distal lower limb muscle weakness (HP:0009053)
-
Maeve Soen
00399563
0000292670
see paper; ..., distal lower limb muscle weakness (HP:0009053); pes cavus (HP:0001761); reduced tendon reflexes (HP:0001315); gait disturbance (HP:0001288); distal amyotrophy (HP:0003693)
CMT1X
-
Familial, X-linked
29y
-
15y
distal lower limb muscle weakness (HP:0009053)
-
Maeve Soen
00399564
0000292671
see paper; ..., distal lower limb amyotrophy (HP:0008944); lower limb muscle weakness (HP:0007340); distal lower limb amyotrophy (HP:0008944); areflexia (HP:0001284); pes cavus (HP:0001761); steppage gait (HP:0003376); mixed demyelinating and axonal polyneuropathy (HP:0007327); reduced tendon reflexes (HP:0001315)
CMT1X
-
Familial, X-linked dominant
37y
-
27y
gait disturbance (HP:0001288)
-
Maeve Soen
00399565
0000292672
see paper; ..., muscle weakness (HP:0001324); reduced tendon reflexes (HP:0001315); gait disturbance (HP:0001288); pes cavus (HP:0001761)
CMT1X
-
Familial, X-linked
65y
-
35y
abnormal foot morphology (HP:0001760)
-
Maeve Soen
00399566
0000292673
see paper; ..., muscle weakness (HP:0001324); distal lower limb amyotrophy (HP:0008944); reduced tendon reflexes (HP:0001315); pes cavus (HP:0001761); steppage gait (HP:0003376); areflexia (HP:0001284); split hand HP:0001171; mixed demyelinating and axonal polyneuropathy (HP:0007327)
CMT1X
-
Familial, X-linked dominant
33y-46y
-
16y
gait disturbance (HP:0001288)
-
Maeve Soen
00399569
0000292675
see paper; ..., hearing impairment (HP:0000365); dysesthesia (HP:0012534); muscle weakness (HP:0001324); reduced tendon reflexes (HP:0001315); distal amyotrophy (HP:0003693); areflexia (HP:0001284); clubbing of fingers (HP:0100759); soft tissue swelling of interphalangeal joints (HP:0006162); split hand HP:0001171; pes cavus (HP:0001761); steppage gait (HP:0003376); mixed demyelinating and axonal polyneuropathy (HP:0007327)
CMT1X
-
Familial, X-linked dominant
39y
-
12y
gait disturbance (HP:0001288); frequent falls (HP:0002359)
-
Maeve Soen
00399570
0000292676
see paper; ..., muscle weakness (HP:0001324); distal amyotrophy (HP:0003693); reduced tendon reflexes (HP:0001315); pes cavus (HP:0001761)
CMT1X
-
Familial, X-linked dominant
72y
-
30y
gait disturbance (HP:0001288)
-
Maeve Soen
00399571
0000350346
Sensorimotor neuropathy, Distal sensory impairment, educed motor nerve conduction velocity, positive family history of HSMN (brother and mother, carriers of the same variant p.Arg181Ala)
-
-
Familial, X-linked
60y
-
-
-
-
Andreas Laner
00464284
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