CSTB Protein (AA 1-98, full length) (GST tag)
Quick Overview for CSTB Protein (AA 1-98, full length) (GST tag) (ABIN7479584)
Target
See all CSTB ProteinsProtein Type
Origin
Source
Application
Purity
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Protein Characteristics
- AA 1-98, full length
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Purification tag / Conjugate
- This CSTB protein is labelled with GST tag.
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Sequence
- MMCGAPSATQ PATAETQHIA DQVRSQLEEK ENKKFPVFKA VSFKSQVVAG TNYFIKVHVG DEDFVHLRVF QSLPHENKPL TLSNYQTNKA KHDELTYF
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Restrictions
- For Research Use only
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Format
- Lyophilized
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Buffer
- PBS buffer, 20mM GSH.
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Storage
- 4 °C
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- CSTB (Cystatin B (Stefin B) (CSTB))
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Alternative Name
- Cystatin-B
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Background
- Background: This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Defects in CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1) [MIM:254800]. EPM1 is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The onset, occurring between 6 and 13 years of age, is characterized by convulsions. Myoclonus begins 1 to 5 years later. The twitchings occur predominantly in the proximal muscles of the extremities and are bilaterally symmetrical, although asynchronous. At first small, they become late in the clinical course so violent that the victim is thrown to the floor. Mental deterioration and eventually dementia develop. Synonyms: CPI-B Liver thiol proteinase inhibitor Stefin-B.
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Molecular Weight
- 38.5 kD
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NCBI Accession
- NM_000100
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UniProt
- P04080
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Pathways
- Response to Water Deprivation
Target
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