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Guanylurea+phosphate


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Catalog Number: (10750-190)

Supplier:  Prosci
Description:   LIMP2 Antibody: The lysosomal integral membrane protein 2 (LIMP2) is a heavily glycosylated type III transmembrane protein, the majority of which exists in the lumen of the lysosome and a cytoplasmic domain of approximately 20 amino acids. A deficiency of LIMP2 in mice causes uretic pelvic junction obstruction, deafness, and peripheral neuropathy associated with impaired vesicular trafficking and distribution of apically expressed proteins. More recently, LIMP2 was shown to act as a receptor to bind beta-glucocerebrosidase, the enzyme defective in Gaucher disease, a lysosomal storage disorder. LIMP2-deficient mice showed missorted as well as secreted beta-glucocerebrosidase, suggesting that LIMP2 also functions as the mannose-6-phosphate-independent trafficking receptor.
Catalog Number: (89266-182)

Supplier:  Genetex
Description:   Rabbit polyclonal antibody to PNPO (N-terminal)
Catalog Number: (10665-932)

Supplier:  Bioss
Description:   Pyridoxal kinase belongs to the pyridoxine kinase family and phosphorylates vitamin B6, a step necessary for the conversion of vitamin B6 to pyridoxal 5’ phosphate (PLP), the active form of vitamin B6. PLP acts as a coenzyme and functions to maintain homeostasis. Pyridoxal kinase is a 312-amino acid cytoplasmic protein that may act as a homodimer and is expressed ubiquitously. There are three known isoforms of pyridoxal kinase, and isoform 3 expression is observed in adult testis and spermatozoa. The optimum pH for pyridoxal kinase is between 5.5 and 6.0. PDXK, the gene that encodes the pyridoxal kinase protein, maps to chromosome 21q22.3 and may be a candidate gene for autoimmune polyglandular disease type 1, a genetic disorder that has been mapped to the same region on chromosome 21.
Catalog Number: (76012-348)

Supplier:  Prosci
Description:   The product of this gene belongs to the FGGY kinase family of proteins and encodes glycerol kinase. Glycerol kinase is a key enzyme in the regulation of glycerol uptake and metabolism. It catalyzes the phosphorylation of glycerol by ATP, yielding ADP and glycerol-3-phosphate. Defects in this gene are the cause of glycerol kinase deficiency (GKD). Alternatively spliced transcript variants encoding different isoforms have been identified.
Catalog Number: (103287-338)

Supplier:  Novus Biologicals
Description:   The HDGF2 Antibody from Novus Biologicals is a rabbit polyclonal antibody to HDGF2. This antibody reacts with human. The HDGF2 Antibody has been validated for the following applications: Immunoprecipitation.
Supplier:  TCI America
Description:   CAS Number: 6757-06-8
MDL Number: MFCD00064355
Molecular Formula: C9H14N3O8P
Molecular Weight: 367.16
Purity/Analysis Method: >99.0% (HPLC)
Form: Crystal
Specific rotation [a]20/D: 8 deg (C=2, H2O)
Storage Temperature: 0-10°C
MSDS SDS
Supplier:  Bioss
Description:   The pantothenate kinase (PANK) family of proteins catalyzes the first step in coenzyme A (CoA) biosynthesis. Coenzyme A is an important coenzyme involved in the synthesis and oxidation of fatty acids, as well as the oxidation of pyruvate in the citric acid (Krebs) cycle. Pantothenate kinase 3 (PANK3) is a 370 amino acid member of the pantothenate kinase family that plays a role in the physiological regulation of the intracellular CoA concentration. Localized to the cytoplasm, PANK3 is regulated by feedback inhibition by CoA and its thioesters. PANK3 transfers a phosphate from ATP to pantothenate (Vitamin B5), resulting in formation of 4’-phosphopantothenate. Closely related to its family members, PANK1, PANK2 and PANK4, PANK3 is highly expressed in liver. Pantothenate kinase associated neurodegeneration (PKAN) results from mutations in the gene encoding PANK2, the only mitochondria targeted human PANK.
Supplier:  Biolegend
Description:   Alexa Fluor® 647 anti-mouse/rat XCR1 [ZET]; Isotype: Mouse IgG2b, κ; Reactivity: Mouse, Rat; Apps: FC; Size: 100 μg
Catalog Number: (103332-716)

Supplier:  Novus Biologicals
Description:   The SLC25A3 Antibody from Novus Biologicals is a mouse polyclonal antibody to SLC25A3. This antibody reacts with human. The SLC25A3 Antibody has been validated for the following applications: Western Blot.
Supplier:  Bioss
Description:   Insulin is a pancreatic hormone that regulates glucose and is involved in the synthesis of protein and fat. It increases cell permeability to monosaccharides, amino acids and fatty acids. It accelerates glycolysis, the pentose phosphate cycle, and glycogen synthesis in liver. Heterodimer of a B chain and an A chain linked by two disulfide bonds.Belongs to the insulin family. The insulin-link growth factors, IGF-I and IGF-II (also desinated somatomedin C and multiplication stimulating activator, respectvely), share approximatly 76% sequence identity and are 50% related to pro-insulin.IGF-I and IGF-II are nonglycosylated, single chain proteins of 70 and 76 amino acids in length, respectivelly. IGF-I functions as an autocrine regulator of growth in vaious, whereas the function of IGF-II is less well defined.
Catalog Number: (103272-424)

Supplier:  Novus Biologicals
Description:   The CPS1 Antibody from Novus Biologicals is a rabbit polyclonal antibody to CPS1. This antibody reacts with human, canine. The CPS1 Antibody has been validated for the following applications: Western Blot, Immunohistochemistry, Immunocytochemistry / Immunofluorescence, Immunohistochemistry-Paraffin.
Catalog Number: (10751-418)

Supplier:  Prosci
Description:   DPAGT1 Antibody: The UDP-N-acetylglucosamine-dolichyl-phosphate N-acetyl-glucosaminephosphotransferase (DPAGT1) is an enzyme that catalyzes the first step in the dolichol-linked oligosaccharide pathway for glycoprotein biosynthesis. Mutations in this integral endoplasmic reticulum (ER) membrane protein enzyme belongs to the glycosyltransferase family 4 results in the congenital disorder of glycosylation type Ij with symptoms such as severe hypotonia, medically intractable seizures, mental retardation, microcephaly, and exotropia. Recent experiments have shown that DPAGT1 is a target of the Wnt/beta-catenin signaling pathway, with Wnt3a inducing higher DPAGT1 mRNA expression.
Catalog Number: (89270-112)

Supplier:  Genetex
Description:   Rabbit polyclonal antibody to SLC17A3 (Middle)
Catalog Number: (89267-108)

Supplier:  Genetex
Description:   Rabbit polyclonal antibody to GPD1L (Middle)
Catalog Number: (RL610-601-002)

Supplier:  Rockland Immunochemical
Description:   Anti-Mouse IgG whole molecule antibody is suitable for use in immunoelectrophoresis, western-blot, competitive western-blot, ELISA and competitive ELISA assays. Specific conditions for reactivity and signal detection should be optimized by the end user.

Supplier:  Adipogen
Description:   FGF-23 (Fibroblast growth factor 23) is a regulator of phosphate homeostasis. It upregulates EGR1 expression in the presence of KLBy. Acts directly on the parathyroid to decrease PTH secretion. Regulates the vitamin-D metabolism. Negatively regulates osteoblast differentiation and matrix mineralization. Defects in FGF-23 are the cause of autosomal dominant hypophosphataemic rickets (ADHR) and of hyperphosphatemic familial tumoral calcinosis (HFTC).
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Stock for this item is limited, but may be available in a warehouse close to you. Please make sure that you are logged in to the site so that available stock can be displayed. If the call is still displayed and you need assistance, please call us at 1-800-932-5000.
This product is marked as restricted and can only be purchased by approved Shipping Accounts. If you need further assistance, email VWR Regulatory Department at Regulatory_Affairs@vwr.com
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