human BCKDHB Over-expression AAV

Name 
AAV-h-BCKDHB
Cat No 
AAV-202029
Availability
4-5 weeks
Categories
AAV, Over-Expression

This is an AAV expressing Human BCKDHB.

AAV-202029
AAV-h-BCKDHB

Ready-to-use AAV expressing Human BCKDHB (branched chain keto acid dehydrogenase E1 subunit beta). Available with optional GFP reporter or cell-specific promoter.

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Product Details

AAV Serotype
Available in AAV1, AAV2, AAV3, AAV5, AAV6, AAV8, AAV9, AAV-DJ, AAV-DJ8, AAV-DJ9 and other wildtype/synthetic AAV capsids
AAV ITR
AAV2
Promoter
CMV (default), or choose from 30 different ubiquitous or cell specific promoter
Reporter
none, optional GFP, CFP, YFP, RFP or mCherry
Storage Buffer
PBS/5% Glycerol
Viral Backbone
Recombinant AAV

Gene Reference Data

Alternate Names
BCKDE1B; BCKDH E1-beta; E1B
Description (Vector)
Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different 3' non-coding regions, but encoding the same isoform.
Gene ID
594
Gene Name (Vector)
branched chain keto acid dehydrogenase E1 subunit beta
Gene Symbol
BCKDHB
HGNC ID
HGNC:987
ORF Size (aa)
657
ORF Size (bp)
657 bp
RefSeq ID
NM_183050
RefSeq Synonyms
NM_183050, NM_001318975, NM_000056, BC040139, BC034481,
Species
human
UniGene ID
Hs.654441

About Storage Conditions

All our viral products should be kept at -80°C. At this temperature, the virus will remain stable for 6-12 months (and in some cases, up to 2 years). Once thawed, the product can be stored at 4°C for 2-3 weeks without significant loss of biological activity. We recommend aliquoting your vectors into low protein binding tubes upon receipt. This helps avoid repeated freeze-thaw cycles, as well as prevent loss of virus. To maintain accurate titer, aliquot in at least 20ul per tube.