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Home / Primary Antibodies / Monoclonal Antibodies

Anti-GAA Antibody Picoband® (monoclonal, 2G7)

SKU : BHA21002502

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Mfr.No.	M01548, M01548-10ug, M01548-Biotin, M01548-Cy3, M01548-Dylight488, M01548-Dylight550, M01548-Dylight594, M01548-FITC, M01548-HRP, M01548-APC, M01548-PE, M01548-iFluor647, M01548-carrier-free
Alternative Names	glucosidase alpha,acid
Background	Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alteative splicing results in multiple transcript variants.
Clonality	Monoclonal
Cross Reactivity	No cross-reactivity with other proteins.
Description	Boster Bio Anti-GAA Antibody Picoband® (monoclonal, 2G7) catalog # M01548. Tested in IF, IHC, ICC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Weste blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.
Function	Essential for the degradation of glycogen in lysosomes. Has highest activity on alpha-1,4-linked glycosidic linkages, but can also hydrolyze alpha-1,6-linkedglucans.
Host	Mouse
Immunogen Region	A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.
Isotype	Mouse IgG2b
Molecular Weight	110 kDa, 95 kDa, 76 kDa
Reactivity	Human
Reconstitution	Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
Specificity	No cross reactivity with other proteins.
Synonyms	Lysosomal alpha-glucosidase;3.2.1.20
Tissue Specificity	Expressed on platelets and leukocytes and is primarily concentrated at the borders between endothelial cells. Expressed in human umbilical vein endothelial cells (HUVECs) (at protein level). Expressed on neutrophils (at protein level). Isoform Long predominates in all tissues examined. Isoform Delta12 is detected only in trachea. Isoform Delta14-15 is only detected in lung. Isoform Delta14 is detected in all tissues examined with the strongest expression in heart. Isoform Delta15 is expressed in brain, testis, ovary, cell surface of platelets, human umbilical vein endothelial cells (HUVECs), Jurkat T-cell leukemia, human erythroleukemia (HEL) and U-937 histiocytic lymphoma cell lines (at protein level).
Note	Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

Uniprot	P10253
Gene Id	2548
Gene Name	GAA
Clone No	Clone: 2G7
Subcellular Location	Lysosome

Conjugates	Biotin, Cy3, Dylight488, Dylight550, Dylight594, FITC, HRP, APC, PE, iFluor647, carrier-free

Concentration	Adding 0.2 ml of distilled water will yield a concentration of 500 ug/ml.
Form	Lyophilized
Purification	Immunogen affinity purified.
Storage	Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Applications	IF, IHC, ICC, WB
Description	Weste blot, 0.1-0.5ug/ml, Human<br> Immunohistochemistry (Paraffin-embedded Section), 0.5-1ug/ml, Human<br> Immunocytochemistry/Immunofluorescence, 2ug/ml, Human<br>

Reference	1. "Entrez Gene: GAA glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)".<br /><br />2. Donald J. Voet; Judith G. Voet; Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538.<br /><br />3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S619.<br /><br />

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Combination SKU	Supplier No.	Conjugation	Size	Your price	Quantity
BHA21002502-1	M01548	-	100 ug	$370		Add to Cart
BHA21002502-2	M01548-10ug	Sample	10 ug	$99		Add to Cart
BHA21002502-3	M01548-Biotin	Biotin	100 ug	$570		Add to Cart
BHA21002502-4	M01548-Cy3	Cy3	100 ug	$570		Add to Cart
BHA21002502-5	M01548-Dylight488	Dylight488	100 ug	$570		Add to Cart
BHA21002502-6	M01548-Dylight550	Dylight550	100 ug	$570		Add to Cart
BHA21002502-7	M01548-Dylight594	Dylight594	100 ug	$570		Add to Cart
BHA21002502-8	M01548-FITC	FITC	100 ug	$570		Add to Cart
BHA21002502-9	M01548-HRP	HRP	100 ug	$570		Add to Cart
BHA21002502-10	M01548-APC	APC	100 ug	$670		Add to Cart
BHA21002502-11	M01548-PE	PE	100 ug	$670		Add to Cart
BHA21002502-12	M01548-iFluor647	iFluor647	100 ug	$670		Add to Cart
BHA21002502-13	M01548-carrier-free	carrier-free	100 ug	$370		Add to Cart

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