Lipoprotein glomerulopathy

General Information (adopted from Orphanet):

Synonyms, Signs: LPG
Number of Symptoms 4
OrphanetNr: 329481
OMIM Id: 611771
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant
[Orphanet]
Age of onset: All ages
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Primary glomerular disease
 -Rare genetic disease
 -Rare renal disease
Rare syndromic dyslipidemia
 -Rare endocrine disease
 -Rare genetic disease

Symptom Information: Sort by abundance 

1
(HPO:0100820) Glomerulopathy 18077821 IBIS 46 / 7739
2
(HPO:0001966) Mesangial abnormality 18077821 IBIS 1 / 7739
3
(HPO:0000093) Proteinuria 18077821 IBIS 169 / 7739
4
(HPO:0000083) Renal insufficiency 18077821 IBIS 232 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) Lipoprotein glomerulopathy is an uncommon kidney disease characterized by proteinuria, progressive kidney failure, and distinctive lipoprotein thrombi in glomerular capillaries (Saito et al., 2006). It mainly affects people of Japanese and Chinese origin; in these populations, it is ...
Clinical Description OMIM Lipoprotein glomerulopathy is characterized by abnormal lipoprotein deposition in the glomeruli, usually with lipoprotein thrombi distending and occluding the glomerular capillary lumina, a variable degree of mesangial proliferation, dysbetalipoproteinemia, and high levels of APOE and APOE2/3 phenotype in ...
Molecular genetics OMIM Oikawa et al. (1997) identified 3 Japanese patients with lipoprotein glomerulopathy who were heterozygous for an arg145-to-pro mutation in APOE (R145P; 107741.0032). The authors designated this variant 'APOE Sendai.'

Matsunaga et al. (1999) reported a Japanese ...