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一般說明
Progranulin (PGRN) is a cysteine-rich protein with 593-amino acids and has a molecular weight of ~68.5kDa. The PGRN gene, with one non-coding exon and 12 coding exons spanning 8kb of genomic DNA, is mapped to human chromosome 17q21. The encoded protein is characterized by seven granulin-like domains, which include 7.5 highly conserved tandem repeats of a rare 12 cysteinyl residues. PGRN is predominantly expressed in rapidly cycling epithelial cells, leukocytes, neurons, and chondrocytes.
生化／生理作用
Progranulin (PGRN) is a widely expressed pluripotent growth factor, which plays a crucial role in various processes such as development, cell cycle progression, cell motility, wound repair and inflammation. Additionally, It is also implicated in early embryogenesis, host defense, cartilage development and degradation. Mutations in the PGRN gene were found in cases of frontotemporal degeneration (FTLD). PGRN expression is associated with the development of various type of cancer such as breast cancer, ovarian carcinoma, and multiple myeloma. Overexpression of PGRN has been observed in neurodegenerative diseases such as microglia of Creutzfeldt-Jacob patients and in spinal cord of amyotrophic lateral sclerosis (ALS) patients.
外觀
Lyophilized from 0.2 μm-filtered solution in PBS.
重構
Reconstitute with 100 μL deionized water.
其他說明
Signal peptide and human progranulin (aa 1-593) is untagged. Reflects the native sequence with no additional aa.
儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 2
閃點（°F）
Not applicable
閃點（°C）
Not applicable
Mutations other than null mutations producing a pathogenic loss of progranulin in frontotemporal dementia.
van der Zee J
Human Mutation, 28(4), 416-416 (2007)
Progranulin in neurodegenerative disease.
Petkau TL and Leavitt BR2
Trends in Neurosciences, 37(7), 388-398 (2014)
Progranulin in frontotemporal lobar degeneration and neuroinflammation.
Ahmed Z
Journal of Neuroinflammation (2007)
The growth factor progranulin binds to TNF receptors and is therapeutic against inflammatory arthritis in mice.
Tang W
Science, 332(6028), 478-484 (2011)
Lorenz Thurner et al.
Digestive diseases and sciences, 59(8), 1733-1742 (2014-03-05)
Recently, we identified neutralizing autoantibodies against progranulin (PGRN) in a wide spectrum of rheumatic diseases including cases with enteropathic spondylarthritis. PGRN is a secreted protein with strong anti-inflammatory effects, believed to be mediated by the direct inhibition of TNF receptors
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