PYCR1, Polyclonal Antibody

Pyrroline-5-carboxylate reductase 1; mitochondrial; P5C reductase 1; P5CR 1; PYCR1

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This PYCR1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 291-319 amino acids from the C-terminal region of human PYCR1.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-PYCR1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene encodes an enzyme that catalyzes the NAD(P)H-dependent conversion of pyrroline-5-carboxylate to proline. This enzyme may also play a physiologic role in the generation of NADP(+) in some cell types. The protein forms a homopolymer and localizes to the mitochondrion.

Metabolism; Signal Transduction

Flow Cytometry (FC/FACS), ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

WB~~1:1000

PYCR1 Antibody (C-term) western blot analysis in K562,WiDr,HepG2 cell line lysates (35ug/lane).This demonstrates the PYCR1 antibody detected the PYCR1 protein (arrow).

PYCR1 Antibody (C-term) IHC analysis in formalin fixed and paraffin embedded colon carcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the PYCR1 Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.

PYCR1 Antibody (C-term) flow cytometric analysis of HepG2 cells (right histogram) compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

33361

pyrroline-5-carboxylate reductase 1

pyrroline-5-carboxylate reductase 1, mitochondrial

Pyrroline-5-carboxylate reductase 1, mitochondrial

P5C reductase 1; P5CR 1  [Similar Products]

P5CR1_HUMAN

This gene encodes an enzyme that catalyzes the NAD(P)H-dependent conversion of pyrroline-5-carboxylate to proline. This enzyme may also play a physiologic role in the generation of NADP(+) in some cell types. The protein forms a homopolymer and localizes to the mitochondrion. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2013]

PYCR1: Housekeeping enzyme that catalyzes the last step in proline biosynthesis. Can utilize both NAD and NADP, but has higher affinity for NAD. Involved in the cellular response to oxidative stress. Defects in PYCR1 are the cause of cutis laxa autosomal recessive type 2B (ARCL2B). A multisystem disorder characterized by the appearance of premature aging, wrinkled and lax skin with reduced elasticity, joint laxity, craniofacial dysmorphic features, intrauterine growth retardation with some degree of postnatal growth deficiency, and developmental delay. Defects in PYCR1 are the cause of cutis laxa, autosomal recessive, type 3B (ARCL3B). ARCL3B is a disorder characterized by an aged appearance with distinctive facial features, sparse hair, ophthalmologic abnormalities, intrauterine growth retardation, and cutis laxa. Belongs to the pyrroline-5-carboxylate reductase family.

Protein type: Oxidoreductase; Amino Acid Metabolism - arginine and proline; EC 1.5.1.2; Mitochondrial

Chromosomal Location of Human Ortholog: 17q25.3

Cellular Component: mitochondrion; mitochondrial matrix

Molecular Function: identical protein binding; protein binding; pyrroline-5-carboxylate reductase activity

Biological Process: regulation of mitochondrial membrane potential; proline biosynthetic process; amino acid biosynthetic process

Disease: Cutis Laxa, Autosomal Recessive, Type Iib; Cutis Laxa, Autosomal Recessive, Type Iiib

Reversade, B., et al. Nat. Genet. 41(9):1016-1021(2009)
Guernsey, D.L., et al. Am. J. Hum. Genet. 85(1):120-129(2009)
Meng, Z., et al. J. Mol. Biol. 359(5):1364-1377(2006)

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