Search Thermo Fisher Scientific
Disclaimer
Clicking the images or links will redirect you to a website hosted by BenchSci that provides third-party scientific content. Neither the content nor the BenchSci technology and processes for selection have been evaluated by us; we are providing them as-is and without warranty of any kind, including for use or application of the Thermo Fisher Scientific products presented.
Product Details
14367-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Published species
Zebrafish
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
GAA Fusion Protein Ag5690 (601-952 aa encoded by BC040431)
Conjugate
Form
Liquid
Concentration
0.16 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
Immunogen sequence: STFAGHGRY AGHWTGDVWS SWEQLASSVP EILQFNLLGV PLVGADVCGF LGNTSEELCV RWTQLGAFYP FMRNHNSLLS LPQEPYSFSE PAQQAMRKAL TLRYALLPHL YTLFHQAHVA GETVARPLFL EFPKDSSTWT VDHQLLWGEA LLITPVLQAG KAEVTGYFPL GTWYDLQTVP VEALGSLPPP PAAPREPAIH SEGQWVTLPA PLDTINVHLR AGYIIPLQGP GLTTTESRQQ PMALAVALTK GGEARGELFW DDGESLEVLE RGAYTQVIFL ARNNTIVNEL VRVTSEGAGL QLQKVTVLGV ATAPQQVLSN GVPVSNFTYS PDTKVLDICV SLLMGEQFLV SWC (601-952 aa encoded by BC040431)
Target Information
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Bioinformatics
Protein Aliases: acid (Pompe disease, glycogen storage disease type II); acid alpha-glucosidase; Acid maltase; Aglucosidase alfa; glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II); Lysosomal alpha-glucosidase
Gene Aliases: E430018M07Rik; GAA; LYAG
UniProt ID: (Human) P10253, (Mouse) P70699, (Rat) Q6P7A9
Entrez Gene ID: (Human) 2548, (Mouse) 14387, (Rat) 367562
maltose metabolic process
regulation of the force of heart contraction
diaphragm contraction
heart morphogenesis
glycogen catabolic process
sucrose metabolic process
glucose metabolic process
lysosome organization
locomotory behavior
tissue development
vacuolar sequestering
muscle cell cellular homeostasis
neuromuscular process controlling posture
neuromuscular process controlling balance
cardiac muscle contraction
carbohydrate metabolic process
glycogen metabolic process
striated muscle contraction
metabolic process
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
Learn more
We're here to help
Get expert recommendations for common problems or connect directly with an on staff expert for technical assistance related to applications, equipment and general product use.
Contact tech support