Abnova

GAA Polyclonal Antibody, MaxPab™

图: 1 / 1

GAA Antibody (H00002548-B01P) in WB

Western Blot analysis of GAA expression in transfected 293T cell line (H00002548-T01) by GAA MaxPab polyclonal antibody.

Lane 1: GAA transfected lysate(104.72 KDa).
Lane 2: Non-transfected lysate.

Product Image Gallery

产品信息

H00002548-B01P

应用

建议稀释比

已发表文章

免疫印迹 (WB)

1:500-1:1,000

产品规格

种属反应

Human

宿主/亚型

Mouse / IgG

分类

Polyclonal

类型

Antibody

抗原

GAA (P10253, 1 a.a. approximately 952 a.a) full-length human protein.

查看免疫原

偶联物

Unconjugated Unconjugated Unconjugated

形式

Liquid

浓度

See Label

纯化类型

Affinity chromatography

保存液

PBS, pH 7.4

内含物

no preservative

保存条件

-20° C, Avoid Freeze/Thaw Cycles

运输条件

Ambient (domestic); Wet ice (international)

产品详细信息

Sequence of this protein is as follows: MGVRHPPCSH RLLAVCALVS LATAALLGHI LLHDFLLVPR ELSGSSPVLE ETHPAHQQGA SRPGPRDAQA HPGRPRAVPT QCDVPPNSRF DCAPDKAITQ EQCEARGCCY IPAKQGLQGA QMGQPWCFFP PSYPSYKLEN LSSSEMGYTA TLTRTTPTFF PKDILTLRLD VMMETENRLH FTIKDPANRR YEVPLETPRV HSRAPSPLYS VEFSEEPFGV IVHRQLDGRV LLNTTVAPLF FADQFLQLST SLPSQYITGL AEHLSPLMLS TSWTRITLWN RDLAPTPGAN LYGSHPFYLA LEDGGSAHGV FLLNSNAMDV VLQPSPALSW RSTGGILDVY IFLGPEPKSV VQQYLDVVGY PFMPPYWGLG FHLCRWGYSS TAITRQVVEN MTRAHFPLDV QWNDLDYMDS RRDFTFNKDG FRDFPAMVQE LHQGGRRYMM IVDPAISSSG PAGSYRPYDE GLRRGVFITN ETGQPLIGKV WPGSTAFPDF TNPTALAWWE DMVAEFHDQV PFDGMWIDMN EPSNFIRGSE DGCPNNELEN PPYVPGVVGG TLQAATICAS SHQFLSTHYN LHNLYGLTEA IASHRALVKA RGTRPFVISR STFAGHGRYA GHWTGDVWSS WEQLASSVPE ILQFNLLGVP LVGADVCGFL GNTSEELCVR WTQLGAFYPF MRNHNSLLSL PQEPYSFSEP AQQAMRKALT LRYALLPHLY TLFHQAHVAG ETVARPLFLE FPKDSSTWTV DHQLLWGEAL LITPVLQAGK AEVTGYFPLG TWYDLQTVPI EALGSLPPPP AAPREPAIHS EGQWVTLPAP LDTINVHLRA GYIIPLQGPG LTTTESRQQP MALAVALTKG GEARGELFWD DGESLEVLER GAYTQVIFLA RNNTIVNELV RVTSEGAGLQ LQKVTVLGVA TAPQQVLSNG VPVSNFTYSP DTKVLDICVS LLMGEQFLVS WC

靶标信息

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.

仅用于科研。不用于诊断过程。未经明确授权不得转售。