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Home / Primary Antibodies / Polyclonal Antibodies

Anti-P5CS/ALDH18A1 Antibody Picoband®

SKU : BHA21002489

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Mfr.No.	A06119-2, A06119-2-10ug, A06119-2-Biotin, A06119-2-Cy3, A06119-2-Dylight488, A06119-2-Dylight550, A06119-2-Dylight594, A06119-2-FITC, A06119-2-HRP, A06119-2-APC, A06119-2-PE, A06119-2-iFluor647, A06119-2-carrier-free
Alternative Names	aldehyde dehydrogenase 18 family member A1
Background	Delta-1-pyrroline-5-carboxylate synthetase (P5CS) is an enzyme that in humans is encoded by the ALDH18A1 gene. This gene is mapped to 10q24.1. This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, oithine and arginine. Mutations in this gene lead to hyperammonemia, hypooithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alteatively spliced transcript variants, encoding different isoforms, have been described for this gene.
Clonality	Polyclonal
Cross Reactivity	No cross-reactivity with other proteins.
Description	Boster Bio Anti-P5CS/ALDH18A1 Antibody Picoband® catalog # A06119-2. Tested in ELISA, WB applications. This antibody reacts with Human, Mouse, Rat. 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	Bifunctional enzyme that converts glutamate to glutamate 5-semialdehyde, an intermediate in the biosynthesis of proline, oithine and arginine.
Host	Rabbit
Immunogen Region	E.coli-derived human P5CS/ALDH18A1 recombinant protein (Position: M1-A224).
Isotype	Rabbit IgG
Molecular Weight	87 kDa
Reactivity	Human, Mouse, Rat
Reconstitution	Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
Specificity	No cross reactivity with other proteins.
Synonyms	Delta-1-pyrroline-5-carboxylate synthase; P5CS; Aldehyde dehydrogenase family 18 member A1; GK; Gamma-glutamyl kinase; GPR; Glutamate-5-semialdehyde dehydrogenase; Glutamyl-gamma-semialdehyde dehydrogenase; ALDH18A1; GSAS; P5CS; PYCS
Tissue Specificity	Detected in pancreas islets (at protein level). Detected in brain and pancreas.
Note	Each vial contains 4mg Trehalose, 0.9mg NaCl, 0.2mg Na2HPO4, 0.01mg NaN3.

Uniprot	P54886
Gene Id	5832
Gene Name	ALDH18A1
Subcellular Location	Mitochondrion inner membrane.

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	ELISA, WB
Description	Weste blot, 0.25-0.5ug/ml, Human, Mouse, Rat<br> ELISA, 0.1-0.5ug/ml, -<br>

Reference	1. Bicknell, L. S., Pitt, J., Aftimos, S., Ramadas, R., Maw, M. A., Robertson, S. P. A missense mutation in ALDH18A1, encoding delta-1-pyrroline-5-carboxylate synthase (P5CS), causes an autosomal recessive neurocutaneous syndrome. Europ. J. Hum. Genet. 16: 1176-1186, 2008.<br /><br />2. Fischer, B., Callewaert, B., Schroter, P., Coucke, P. J., Schlack, C., Ott, C.-E., Morroni, M., Homann, W., Mundlos, S., Morava, E., Ficcadenti, A., Koak, U. Severe congenital cutis laxa with cardiovascular manifestations due to homozygous deletions in ALDH18A1. Molec. Genet. Metab. 112: 310-316, 2014.<br /><br />3. Panza, E., Escamilla-Honrubia, J. M., Marco-Marin, C., Gougeard, N., De Michele, G., Brescia Morra, V., Liguori, R., Salviati, L., Donati, M. A., Cusano, R., Pippucci, T., Ravazzolo, R., Nemeth, A. H., Smithson, S., Davies, S., Hurst, J. A., Bordo, D., Rubio, V., Seri, M. ALDH18A1 gene mutations cause dominant spastic paraplegia SPG9: loss of function effect and plausibility of a dominant negative mechanism. (Letter) Brain 139: e3, 2016. Note: Electronic Article.

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Combination SKU	Supplier No.	Conjugation	Size	Your price	Quantity
BHA21002489-1	A06119-2	-	100 ug	$370		Add to Cart
BHA21002489-2	A06119-2-10ug	Sample	10 ug	$99		Add to Cart
BHA21002489-3	A06119-2-Biotin	Biotin	100 ug	$570		Add to Cart
BHA21002489-4	A06119-2-Cy3	Cy3	100 ug	$570		Add to Cart
BHA21002489-5	A06119-2-Dylight488	Dylight488	100 ug	$570		Add to Cart
BHA21002489-6	A06119-2-Dylight550	Dylight550	100 ug	$570		Add to Cart
BHA21002489-7	A06119-2-Dylight594	Dylight594	100 ug	$570		Add to Cart
BHA21002489-8	A06119-2-FITC	FITC	100 ug	$570		Add to Cart
BHA21002489-9	A06119-2-HRP	HRP	100 ug	$570		Add to Cart
BHA21002489-10	A06119-2-APC	APC	100 ug	$670		Add to Cart
BHA21002489-11	A06119-2-PE	PE	100 ug	$670		Add to Cart
BHA21002489-12	A06119-2-iFluor647	iFluor647	100 ug	$670		Add to Cart
BHA21002489-13	A06119-2-carrier-free	carrier-free	100 ug	$370		Add to Cart

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