ALMS
MCID: ALS001
MIFTS: 53

Alstrom Syndrome (ALMS) malady

Categories: Genetic diseases, Rare diseases, Eye diseases, Nephrological diseases, Ear diseases, Endocrine diseases, Fetal diseases

Aliases & Classifications for Alstrom Syndrome

Aliases & Descriptions for Alstrom Syndrome:

Name: Alstrom Syndrome 54 12 23 50 24 25 66 13 52 42 14 69 23 50 25 56
Alms 50 25 66
Alstrom's Syndrome 50 29
Alstrom-Hallgren Syndrome 25
Alstroem Syndrome 66
Alss 50

Characteristics:

Orphanet epidemiological data:

56
alström syndrome
Inheritance: Autosomal recessive; Prevalence: 1-9/1000000 (Worldwide); Age of onset: Adolescent,Adult,Childhood,Infancy,Neonatal; Age of death: adolescent,adult,early childhood,infantile,late childhood,young Adult;

GeneReviews:

23
alstrom syndrome:
Mortality/Aging death in early adulthood
Inheritance autosomal recessive inheritance


GeneReviews:

23
Penetrance No clinically unaffected individuals with two alms1 pathogenic variants in trans have been reported; thus, penetrance appears to be 100% [marshall et al 2011a]...

Classifications:



External Ids:

OMIM 54 203800
Disease Ontology 12 DOID:0050473
MeSH 42 D056769
NCIt 47 C84549
SNOMED-CT 64 63702009
Orphanet 56 ORPHA64
MESH via Orphanet 43 D056769
UMLS via Orphanet 70 C0268425
MedGen 40 C0268425
UMLS 69 C0268425

Summaries for Alstrom Syndrome

NIH Rare Diseases : 50 alström syndrome is a rare genetic disorder that affects many body systems. symptoms develop gradually, beginning in infancy, and can be variable. in childhood, the disorder is generally characterized by vision and hearing abnormalities, childhood obesity, and heart disease (cardiomyopathy). over time, diabetes mellitus, liver problems, and slowly progressive kidney dysfunction which can lead to kidney failure may develop. alström syndrome is caused by mutations in the alms1 gene. it is inherited in an autosomal recessive manner. while there is no specific treatment for alström syndrome, symptoms can be managed by a team of specialists with the goal of improving the quality of life and increasing the lifespan. last updated: 6/22/2016

MalaCards based summary : Alstrom Syndrome, also known as alms, is related to megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome 2 and pnpla6-related disorders, and has symptoms including seizures, photophobia and nystagmus. An important gene associated with Alstrom Syndrome is ALMS1 (ALMS1, Centrosome And Basal Body Associated Protein). The drug Liver Extracts has been mentioned in the context of this disorder. Affiliated tissues include kidney, heart and liver, and related phenotypes are Increased shRNA abundance (Z-score > 2) and cardiovascular system

Disease Ontology : 12 An autosomal recessive disease that is characterized by multiorgan dysfunction. The key features are childhood obesity, blindness due to congenital retinal dystrophy, and sensorineural hearing loss with autosomal recessive inheritance and has material basis in mutations in the ALMS1 gene.

OMIM : 54 Alstrom syndrome is an autosomal recessive disorder characterized by progressive cone-rod dystrophy leading to... (203800) more...

UniProtKB/Swiss-Prot : 66 Alstrom syndrome: A rare autosomal recessive disorder characterized by progressive cone- rod retinal dystrophy, neurosensory hearing loss, early childhood obesity and diabetes mellitus type 2. Dilated cardiomyopathy, acanthosis nigricans, male hypogonadism, hypothyroidism, developmental delay and hepatic dysfunction can also be associated with the syndrome.

Wikipedia : 71 Alström syndrome, also called Alstrom-Halgren Syndrome, is a rare genetic disorder caused by mutations... more...

GeneReviews: NBK1267

Related Diseases for Alstrom Syndrome

Graphical network of the top 20 diseases related to Alstrom Syndrome:



Diseases related to Alstrom Syndrome

Symptoms & Phenotypes for Alstrom Syndrome

Symptoms by clinical synopsis from OMIM:

203800

Clinical features from OMIM:

203800

Human phenotypes related to Alstrom Syndrome:

56 32 (show top 50) (show all 83)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 seizures 56 32 Occasional (29-5%) HP:0001250
2 photophobia 56 32 Frequent (79-30%) HP:0000613
3 nystagmus 56 32 Frequent (79-30%) HP:0000639
4 depression 56 32 Occasional (29-5%) HP:0000716
5 hypothyroidism 56 32 Occasional (29-5%) HP:0000821
6 precocious puberty 56 32 Occasional (29-5%) HP:0000826
7 intellectual disability 56 32 Occasional (29-5%) HP:0001249
8 respiratory insufficiency 56 32 Frequent (79-30%) HP:0002093
9 chronic otitis media 56 32 Frequent (79-30%) HP:0000389
10 cataract 56 32 Frequent (79-30%) HP:0000518
11 global developmental delay 56 32 Occasional (29-5%) HP:0001263
12 splenomegaly 56 32 Occasional (29-5%) HP:0001744
13 recurrent respiratory infections 56 32 Frequent (79-30%) HP:0002205
14 hepatomegaly 56 32 Occasional (29-5%) HP:0002240
15 type ii diabetes mellitus 56 32 Frequent (79-30%) HP:0005978
16 short stature 56 32 Very frequent (99-80%) HP:0004322
17 blindness 56 32 Frequent (79-30%) HP:0000618
18 renal insufficiency 56 32 Occasional (29-5%) HP:0000083
19 portal hypertension 56 32 Occasional (29-5%) HP:0001409
20 pulmonary fibrosis 56 32 Occasional (29-5%) HP:0002206
21 hypertriglyceridemia 56 32 Frequent (79-30%) HP:0002155
22 acanthosis nigricans 56 32 Frequent (79-30%) HP:0000956
23 congestive heart failure 56 32 Occasional (29-5%) HP:0001635
24 generalized hirsutism 56 32 Occasional (29-5%) HP:0002230
25 progressive visual loss 56 32 Frequent (79-30%) HP:0000529
26 autism 56 32 Occasional (29-5%) HP:0000717
27 hepatic steatosis 56 32 Occasional (29-5%) HP:0001397
28 hyperinsulinemia 56 32 Frequent (79-30%) HP:0000842
29 cirrhosis 56 32 Occasional (29-5%) HP:0001394
30 aplasia/hypoplasia of the cerebellum 56 32 Occasional (29-5%) HP:0007360
31 glomerulopathy 56 32 Occasional (29-5%) HP:0100820
32 male hypogonadism 56 32 Occasional (29-5%) HP:0000026
33 deeply set eye 56 32 Occasional (29-5%) HP:0000490
34 decreased testicular size 56 32 Occasional (29-5%) HP:0008734
35 polycystic ovaries 56 32 Occasional (29-5%) HP:0000147
36 round face 56 32 Occasional (29-5%) HP:0000311
37 vesicoureteral reflux 56 32 Occasional (29-5%) HP:0000076
38 asthma 56 32 Frequent (79-30%) HP:0002099
39 nephrocalcinosis 56 32 Occasional (29-5%) HP:0000121
40 obsessive-compulsive behavior 56 32 Occasional (29-5%) HP:0000722
41 alopecia 56 32 Occasional (29-5%) HP:0001596
42 renovascular hypertension 56 32 Occasional (29-5%) HP:0100817
43 chorioretinal abnormality 56 32 Very frequent (99-80%) HP:0000532
44 abnormality of the urethra 56 32 Occasional (29-5%) HP:0000795
45 dilated cardiomyopathy 56 32 Frequent (79-30%) HP:0001644
46 truncal obesity 56 32 Very frequent (99-80%) HP:0001956
47 abnormality of female external genitalia 56 32 Occasional (29-5%) HP:0000055
48 tubulointerstitial nephritis 56 32 Occasional (29-5%) HP:0001970
49 insulin resistance 56 32 Frequent (79-30%) HP:0000855
50 chronic hepatic failure 56 32 Occasional (29-5%) HP:0100626

GenomeRNAi Phenotypes related to Alstrom Syndrome according to GeneCards Suite gene sharing:

26
id Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.72 RPGR
2 Increased shRNA abundance (Z-score > 2) GR00366-A-107 9.72 NODAL
3 Increased shRNA abundance (Z-score > 2) GR00366-A-121 9.72 NODAL
4 Increased shRNA abundance (Z-score > 2) GR00366-A-147 9.72 DLX3 NODAL
5 Increased shRNA abundance (Z-score > 2) GR00366-A-149 9.72 RPGR
6 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.72 TGFA
7 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.72 NODAL
8 Increased shRNA abundance (Z-score > 2) GR00366-A-164 9.72 DLX3
9 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.72 RPGR TGFA DLX3 NODAL
10 Increased shRNA abundance (Z-score > 2) GR00366-A-189 9.72 NODAL
11 Increased shRNA abundance (Z-score > 2) GR00366-A-202 9.72 NODAL
12 Increased shRNA abundance (Z-score > 2) GR00366-A-207 9.72 RPGR
13 Increased shRNA abundance (Z-score > 2) GR00366-A-22 9.72 TGFA
14 Increased shRNA abundance (Z-score > 2) GR00366-A-4 9.72 DLX3
15 Increased shRNA abundance (Z-score > 2) GR00366-A-45 9.72 RPGR
16 Increased shRNA abundance (Z-score > 2) GR00366-A-49 9.72 RPGR
17 Increased shRNA abundance (Z-score > 2) GR00366-A-50 9.72 RPGR
18 Increased shRNA abundance (Z-score > 2) GR00366-A-52 9.72 DLX3
19 Increased shRNA abundance (Z-score > 2) GR00366-A-73 9.72 RPGR
20 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.72 TGFA
21 Increased shRNA abundance (Z-score > 2) GR00366-A-9 9.72 DLX3

MGI Mouse Phenotypes related to Alstrom Syndrome:

44 (show all 11)
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.18 BBS1 DLX3 INS KCNJ11 LEP NODAL
2 growth/size/body region MP:0005378 10.14 ALMS1 BBS1 BBS2 DLX3 INS KCNJ11
3 homeostasis/metabolism MP:0005376 10.11 ALMS1 BBS1 BBS2 DCTN1 INS KCNJ11
4 adipose tissue MP:0005375 10.04 ALMS1 BBS1 BBS2 INS KCNJ11 LEP
5 endocrine/exocrine gland MP:0005379 10.03 TGFA ALMS1 BBS2 DLX3 INS KCNJ11
6 embryo MP:0005380 10 DCTN1 DLX3 INS LBX2 LEP NODAL
7 nervous system MP:0003631 10 RPGR SLC4A4 SLC4A5 BBS1 BBS2 DCTN1
8 muscle MP:0005369 9.87 INS KCNJ11 LEP NODAL RPGR SLC4A4
9 renal/urinary system MP:0005367 9.7 ALMS1 BBS1 BBS2 INS LEP SLC4A4
10 reproductive system MP:0005389 9.56 ALMS1 BBS1 BBS2 INS LEP NODAL
11 vision/eye MP:0005391 9.28 NODAL RPGR SLC4A4 TGFA ALMS1 BBS1

Drugs & Therapeutics for Alstrom Syndrome

Drugs for Alstrom Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


id Name Status Phase Clinical Trials Cas Number PubChem Id
1 Liver Extracts

Interventional clinical trials:


id Name Status NCT ID Phase
1 Setmelanotide Phase 2 Treatment Trial in Patients With Rare Genetic Disorders of Obesity Recruiting NCT03013543 Phase 2, Phase 3
2 Safety and Tolerability of PBI-4050 and Its Effects on the Biomarkers in Subjects With Alström Syndrome Recruiting NCT02739217 Phase 2
3 Clinical Study of a Single Ciliopathy: Alström Syndrome Recruiting NCT02890550
4 Clinical and Molecular Investigations Into Ciliopathies Active, not recruiting NCT00068224

Search NIH Clinical Center for Alstrom Syndrome

Cochrane evidence based reviews: alstrom syndrome

Genetic Tests for Alstrom Syndrome

Genetic tests related to Alstrom Syndrome:

id Genetic test Affiliating Genes
1 Alstrom Syndrome 29 24 ALMS1

Anatomical Context for Alstrom Syndrome

MalaCards organs/tissues related to Alstrom Syndrome:

39
Kidney, Heart, Liver, Eye, Ovary, Cerebellum

Publications for Alstrom Syndrome

Articles related to Alstrom Syndrome:

(show all 29)
id Title Authors Year
1
Characterization of Alstrom Syndrome 1 (ALMS1) Transcript Variants in Hodgkin Lymphoma Cells. ( 28135309 )
2017
2
Metabolic Syndrome in Childhood: Rare Case of Alstrom Syndrome with Blindness. ( 27605748 )
2016
3
Differential effects on I^-cell mass by disruption of Bardet-Biedl syndrome or Alstrom syndrome genes. ( 26494903 )
2015
4
High quality, patient centred and coordinated care for Alstrom syndrome: a model of care for an ultra-rare disease. ( 26603037 )
2015
5
Ophthalmic Features of Children Not Yet Diagnosed with Alstrom Syndrome. ( 25864795 )
2015
6
Atypical Alstrom syndrome with novel ALMS1 mutations precluded by current diagnostic criteria. ( 24503146 )
2014
7
Presentation and course of diabetes in children and adolescents with Alstrom syndrome. ( 21518413 )
2011
8
Alstrom syndrome: A rare genetic disorder and its anaesthetic significance. ( 20661355 )
2010
9
Rare case of Alstrom syndrome with empty sella and interfamilial presence of Bardet-Biedl phenotype. ( 20108498 )
2009
10
Novel human pathological mutations. Gene symbol: ALMS1. Disease: Alstrom syndrome. ( 17879432 )
2007
11
Novel human pathological mutations. Gene symbol: ALMS1. Disease: Alstrom syndrome. ( 17879439 )
2007
12
Alstrom syndrome (OMIM 203800): a case report and literature review. ( 18154657 )
2007
13
Alstrom syndrome in four sibs from northern Jordan. ( 17146208 )
2006
14
Rare case of Alstrom syndrome without obesity and with short stature, diagnosed in adulthood. ( 16669965 )
2006
15
Syndromic obesity and diabetes: changes in body composition with age and mutation analysis of ALMS1 in 12 United Kingdom kindreds with Alstrom syndrome. ( 16720663 )
2006
16
Mutation of ALMS1, a large gene with a tandem repeat encoding 47 amino acids, causes Alstrom syndrome. ( 11941370 )
2002
17
Mutations in ALMS1 cause obesity, type 2 diabetes and neurosensory degeneration in Alstrom syndrome. ( 11941369 )
2002
18
Alstrom syndrome. ( 12038666 )
2002
19
Early-onset liver disease complicated with acute liver failure in Alstrom syndrome. ( 11343329 )
2001
20
Alstrom syndrome: a case report. ( 11732744 )
2001
21
Alstrom syndrome in two siblings. ( 11265260 )
2001
22
Characterization of the murine Lbx2 promoter, identification of the human homologue, and evaluation as a candidate for Alstrom syndrome. ( 11386758 )
2001
23
Alstrom syndrome with hepatic dysfunction: report of one case. ( 11100527 )
2000
24
Alstrom syndrome: confirmation of linkage to chromosome 2p12-13 and phenotypic heterogeneity in three affected sibs. ( 10882760 )
2000
25
Acute lymphoblastic leukemia in one of two siblings with Alstrom syndrome. ( 11061078 )
2000
26
TGFA: exon-intron structure and evaluation as a candidate gene for Alstrom syndrome. ( 10066034 )
1999
27
Human DCTN1: genomic structure and evaluation as a candidate for Alstrom syndrome. ( 9799602 )
1998
28
The Alstrom syndrome: a new variant? ( 8181924 )
1994
29
Alstrom syndrome. ( 1800358 )
1991

Variations for Alstrom Syndrome

ClinVar genetic disease variations for Alstrom Syndrome:

6 (show all 25)
id Gene Variation Type Significance SNP ID Assembly Location
1 ALMS1 ALMS1, 19-BP INS insertion Pathogenic
2 ALMS1 NM_015120.4(ALMS1): c.8383C> T (p.Gln2795Ter) single nucleotide variant Pathogenic rs193919338 GRCh37 Chromosome 2, 73717466: 73717466
3 ALMS1 NM_015120.4(ALMS1): c.10775delC (p.Thr3592Lysfs) deletion Pathogenic rs387906312 GRCh37 Chromosome 2, 73799776: 73799776
4 ALMS1 NM_015120.4(ALMS1): c.2141_2142delCT (p.Ser714Tyrfs) deletion Pathogenic rs387906313 GRCh37 Chromosome 2, 73675792: 73675793
5 ALMS1 NM_015120.4(ALMS1): c.10992G> A (p.Trp3664Ter) single nucleotide variant Pathogenic rs193919339 GRCh37 Chromosome 2, 73799993: 73799993
6 ALMS1 NM_015120.4(ALMS1): c.8164C> T (p.Arg2722Ter) single nucleotide variant Pathogenic rs193919340 GRCh37 Chromosome 2, 73717247: 73717247
7 ALMS1 ALMS1, 333-BP ALU INS, EX16 insertion Pathogenic
8 ALMS1 NM_015120.4(ALMS1): c.10945G> T (p.Glu3649Ter) single nucleotide variant Pathogenic rs397514576 GRCh37 Chromosome 2, 73799946: 73799946
9 ALMS1 NM_015120.4(ALMS1): c.1794_1801dupGGCTTTGA (p.Lys601Argfs) duplication Pathogenic rs398122991 GRCh37 Chromosome 2, 73675445: 73675452
10 ALMS1 NM_015120.4(ALMS1): c.11116_11134del19 (p.Arg3706Leufs) deletion Pathogenic rs398122992 GRCh37 Chromosome 2, 73800117: 73800135
11 ALMS1 NM_015120.4(ALMS1): c.4296_4299delCACA (p.His1432Glnfs) deletion Pathogenic rs398122993 GRCh37 Chromosome 2, 73677947: 73677950
12 ALMS1 NM_015120.4(ALMS1): c.5926delG (p.Glu1976Serfs) deletion Pathogenic rs398122994 GRCh37 Chromosome 2, 73679577: 73679577
13 ALMS1 NM_015120.4(ALMS1): c.1900C> T (p.Gln634Ter) single nucleotide variant Pathogenic rs398122995 GRCh37 Chromosome 2, 73675551: 73675551
14 ALMS1 NM_015120.4(ALMS1): c.4156dupA (p.Thr1386Asnfs) duplication Pathogenic rs797045228 GRCh37 Chromosome 2, 73677807: 73677807
15 ALMS1 NM_015120.4(ALMS1): c.6436C> T (p.Arg2146Ter) single nucleotide variant Pathogenic rs770558150 GRCh37 Chromosome 2, 73680087: 73680087
16 ALMS1 NM_015120.4(ALMS1): c.10483C> T (p.Gln3495Ter) single nucleotide variant Pathogenic rs772624348 GRCh37 Chromosome 2, 73799484: 73799484
17 ALMS1 NM_015120.4(ALMS1): c.5145T> G (p.Tyr1715Ter) single nucleotide variant Likely pathogenic rs772136379 GRCh37 Chromosome 2, 73678796: 73678796
18 ALMS1 NM_015120.4(ALMS1): c.2822T> A (p.Leu941Ter) single nucleotide variant Pathogenic rs539612316 GRCh38 Chromosome 2, 73449346: 73449346
19 ALMS1 NM_015120.4(ALMS1): c.3019dupA (p.Arg1007Lysfs) duplication Pathogenic rs878854998 GRCh38 Chromosome 2, 73449543: 73449543
20 ALMS1 NM_015120.4(ALMS1): c.9433dupA (p.Thr3145Asnfs) duplication Pathogenic rs878855003 GRCh37 Chromosome 2, 73718516: 73718516
21 ALMS1 NM_015120.4(ALMS1): c.5625delA (p.Ala1876Glnfs) deletion Likely pathogenic rs1057517445 GRCh37 Chromosome 2, 73679276: 73679276
22 ALMS1 NM_015120.4(ALMS1): c.7247C> A (p.Ser2416Ter) single nucleotide variant Pathogenic rs1060500039 GRCh38 Chromosome 2, 73453771: 73453771
23 ALMS1 NM_015120.4(ALMS1): c.10303delCinsGA (p.Gln3435Glufs) indel Pathogenic rs1060500034 GRCh38 Chromosome 2, 73559058: 73559058
24 ALMS1 NM_015120.4(ALMS1): c.363_366dupAGTA (p.Tyr123Serfs) duplication Pathogenic GRCh38 Chromosome 2, 73408657: 73408660
25 ALMS1 NM_015120.4(ALMS1): c.5590C> T (p.Gln1864Ter) single nucleotide variant Pathogenic rs769440001 GRCh38 Chromosome 2, 73452114: 73452114

Expression for Alstrom Syndrome

Search GEO for disease gene expression data for Alstrom Syndrome.

Pathways for Alstrom Syndrome

GO Terms for Alstrom Syndrome

Cellular components related to Alstrom Syndrome according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 microtubule organizing center GO:0005815 9.56 BBS1 BBS2 DCTN1 RPGR
2 cilium GO:0005929 9.46 ALMS1 BBS1 BBS2 RPGR
3 BBSome GO:0034464 8.96 BBS1 BBS2
4 ciliary basal body GO:0036064 8.92 ALMS1 BBS1 BBS2 RPGR

Biological processes related to Alstrom Syndrome according to GeneCards Suite gene sharing:

(show all 15)
id Name GO ID Score Top Affiliating Genes
1 glucose homeostasis GO:0042593 9.63 ALMS1 INS LEP
2 cilium assembly GO:0060271 9.56 ALMS1 BBS1 BBS2 RPGR
3 Golgi to plasma membrane protein transport GO:0043001 9.55 BBS1 BBS2
4 positive regulation of mitotic nuclear division GO:0045840 9.54 INS TGFA
5 inorganic anion transport GO:0015698 9.52 SLC4A4 SLC4A5
6 anion transport GO:0006820 9.51 SLC4A4 SLC4A5
7 glucose metabolic process GO:0006006 9.5 INS KCNJ11 LEP
8 melanosome transport GO:0032402 9.49 BBS2 DCTN1
9 regulation of fat cell differentiation GO:0045598 9.48 ALMS1 LEP
10 positive regulation of insulin receptor signaling pathway GO:0046628 9.43 INS LEP
11 negative regulation of multicellular organism growth GO:0040015 9.4 ALMS1 BBS2
12 leptin-mediated signaling pathway GO:0033210 9.37 BBS2 LEP
13 placenta development GO:0001890 9.33 DLX3 LEP NODAL
14 non-motile cilium assembly GO:1905515 9.13 BBS1 BBS2 DCTN1
15 negative regulation of appetite by leptin-mediated signaling pathway GO:0038108 8.62 BBS2 LEP

Molecular functions related to Alstrom Syndrome according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 inorganic anion exchanger activity GO:0005452 9.16 SLC4A4 SLC4A5
2 anion transmembrane transporter activity GO:0008509 8.96 SLC4A4 SLC4A5
3 sodium:bicarbonate symporter activity GO:0008510 8.62 SLC4A4 SLC4A5

Sources for Alstrom Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
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48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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