Anti-AASS Antibody Picoband®

SKU: A07302-3

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,ICC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.1-0.25 μg/ml, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: AASS

Specificity:

Background: Alpha-aminoadipic semialdehyde synthase is an enzyme encoded by the AASS gene in humans and is involved in their major lysine degradation pathway. This gene encodes a bifunctional enzyme that catalyzes the first two steps in the mammalian lysine degradation pathway. The N-terminal and the C-terminal portions of this enzyme contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively, resulting in the conversion of lysine to alpha-aminoadipic semialdehyde. Mutations in this gene are associated with familial hyperlysinemia.

Immunogen: E.coli-derived human AASS recombinant protein (Position: E37-N865).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Dancis, J., Hutzler, J., Woody, N. C., Cox, R. P. Multiple enzyme defects in familial hyperlysinemia. Pediat. Res. 10: 686-691, 1976. 2. Houten, S. M., te Brinke, H., Denis, S., Ruiter, J. P. N., Knegt, A. C., de Klerk, J. B. C., Augoustides-Savvopoulou, P., Haberle, J., Baumgartner, M. R., Coskun, T., Zschocke, J., Sass, J. O., Poll-The, B. T., Wanders, R. J. A., Duran, M. Genetic basis of hyperlysinemia. Orphanet J. Rare Dis. 8: 57, 2013. Note: Electronic Article. 3. Sacksteder, K. A., Biery, B. J., Morrell, J. C., Goodman, B. K., Geisbrecht, B. V., Cox, R. P., Gould, S. J., Geraghty, M. T. Identification of the alpha-aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia. Am. J. Hum. Genet. 66: 1736-1743, 2000.

Uniprot ID: Q9UDR5

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 102 kDa

Calculated Molecular Weight: 108320 MW

Gene ID: 10157

Protein Name: Alpha-aminoadipic semialdehyde synthase, mitochondrial

Gene Full Name: aminoadipate-semialdehyde synthase

Synonyms: aminoadipate-semialdehyde synthase; LKRSDH; LORSDH; LKR/SDH