Immunoglobulin Light Chain Amyloidosis

Alias:
Primary Amyloidosis
Al Amyloidosis
Primary Systemic Amyloidosis
Light-Chain Amyloidosis
Hereditary Amyloid Nephropathy Due to Lysozyme Variant
Familial Amyloid Nephropathy Due to Lysozyme Variant
Hereditary Renal Amyloidosis Due to Lysozyme Variant
Familial Renal Amyloidosis Due to Lysozyme Variant
Immunoglobulin Light-Chain Amyloidosis
Immunoglobulin Deposition Disease
Primary Systemic Al Amyloidosis
Amyloidosis Primary Systemic
Immunoglobulinic Amyloidosis
Light Chain Amyloidosis
Systemic Al Amyloidosis
Primary Al Amyloidosis
Systemic Al Amyloidsis
Lysozyme Amyloidosis
Amyloidosis Primary
Alys Amyloidosis
Amyloid Al
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基础信息
疾病表征
基因 & 突变
靶点药物
疾病模型
文献报道
免疫球蛋白轻链淀粉样变性,也称为原发性淀粉样变性,与原发性皮肤淀粉样变性和多发性骨髓瘤有关。与免疫球蛋白轻链淀粉样变性相关的基因是LYZ(溶菌酶),其相关通路/超通路包括Scavenger受体结合和摄取配体以及Ca、cAMP和脂质信号转导。在该疾病的背景下提到了Ichthammol和Daratumumab药物。附属组织包括肝脏和脾脏,相关表型为疲劳和蛋白尿。
Related ID:
MESH:D000075363
ICD11:1061366491

Basic Information

Inheritance
Age of Onset
Prevalence
Related Gene
Related Mouse Models
Reference
MALACARDS
常显
未知
1-5/10000
21
164
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Medical Symptom

#
Categorization
Description
HPO Frequency
Orphanet Frequency
HPO Source Accession
No data available

Gene & Mutation

#
Gene
Function
Score
Mutations
No data available

Related Drugs

Name
CAS Number
Status
Phase
No data available

Disease Model

Category
Name
MGI
Related Gene
Strain of Origin
Publications
No data available

References Literature

Title
PMID
Journal
Year
IF
No Data Found!
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