protein

Recombinant human Alkaline phosphatase, tissue-nonspecific isozyme

MBS1265592

0.05 mg

195 USD

Recombinant Protein

Recombinant human Alkaline phosphatase, tissue-nonspecific isozyme

Alkaline phosphatase, tissue-nonspecific isozyme

alkaline phosphatase; Alkaline phosphatase, tissue-nonspecific isozyme; alkaline phosphatase, tissue-nonspecific isozyme; glycerophosphatase; tissue-nonspecific ALP; alkaline phosphomonoesterase; liver/bone/kidney-type alkaline phosphatase; alkaline phosphatase liver/bone/kidney isozyme; alkaline phosphatase, liver/bone/kidney; Alkaline phosphatase liver/bone/kidney isozyme

ALPL; ALPL; HOPS; TNAP; APTNAP; TNSALP; AP-TNAP; AP-TNAP; TNSALP

PPBT_HUMAN

E Coli

RARYPGSVLPRACAWTDPRQCSAQDWNIS

Store at -20 degree C. For extended storage, conserve at -20 or -80 degree C.

Tag Information: Host tag may vary. Please inquire for specific tag information.

178462

AAB59378.1

P05186

57,305 Da

AGE/RAGE Pathway 698754!!BDNF Signaling Pathway 712093!!Endochondral Ossification Pathway 198812!!Folate Biosynthesis Pathway 83018!!Folate Biosynthesis Pathway 404!!TNF-alpha/NF-kB Signaling Pathway 198884

There are at least four distinct but related alkaline phosphatases: intestinal, placental, placental-like, and liver/bone/kidney (tissue non-specific). The first three are located together on chromosome 2, while the tissue non-specific form is located on chromosome 1. The product of this gene is a membrane bound glycosylated enzyme that is not expressed in any particular tissue and is, therefore, referred to as the tissue-nonspecific form of the enzyme. The exact physiological function of the alkaline phosphatases is not known. A proposed function of this form of the enzyme is matrix mineralization; however, mice that lack a functional form of this enzyme show normal skeletal development. This enzyme has been linked directly to hypophosphatasia, a disorder that is characterized by hypercalcemia and includes skeletal defects. The character of this disorder can vary, however, depending on the specific mutation since this determines age of onset and severity of symptoms. Alternatively spliced transcript variants have been described. [provided by RefSeq, Apr 2010]

Function: This isozyme may play a role in skeletal mineralization. Catalytic activity: A phosphate monoester + H2O = an alcohol + phosphate. Cofactor: Binds 1 magnesium ion . By similarity.Binds 2 zinc ions . By similarity. Subunit structure: Homodimer. Subcellular location: Cell membrane; Lipid-anchor GPI-anchor. Post-translational modification: Glycosylated. Ref.11. Involvement in disease: Hypophosphatasia (HOPS) [MIM:146300]: A metabolic bone disease characterized by defective skeletal mineralization and biochemically by deficient activity of the tissue non-specific isoenzyme of alkaline phosphatase. Four forms are distinguished, depending on the age of onset: perinatal, infantile, childhood and adult type. The perinatal form is the most severe and is almost always fatal. The adult form is mild and characterized by recurrent fractures, osteomalacia, rickets, and loss of teeth. Some cases are asymptomatic, while some patients manifest dental features without skeletal manifestations (odontohypophosphatasia).Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.4 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21 Ref.22 Ref.23 Ref.24 Ref.25 Ref.26 Ref.27 Ref.28 Ref.29 Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.35 Ref.36 Ref.37 Ref.38Hypophosphatasia childhood type (HOPSC) [MIM:241510]: A bone disease characterized by defective skeletal mineralization and biochemically by deficient activity of the tissue non-specific isoenzyme of alkaline phosphatase.Note: The disease is caused by mutations affecting the gene represented in this entry.Hypophosphatasia infantile type (HOPSI) [MIM:241500]: A severe bone disease characterized by defective skeletal mineralization and biochemically by deficient activity of the tissue non-specific isoenzyme of alkaline phosphatase. Three more or less distinct types of infantile hypophosphatasia can be identified: (1) type 1 with onset in utero or in early postnatal life, craniostenosis, severe skeletal abnormalities, hypercalcemia, and death in the first year or so of life; (2) type 2 with later, more gradual development of symptoms, moderately severe 'rachitic' skeletal changes and premature loss of teeth; (3) type 3 with no symptoms, the condition being determined on routine studies.Note: The disease is caused by mutations affecting the gene represented in this entry. Sequence similarities: Belongs to the alkaline phosphatase family. Sequence caution: The sequence BAD93051.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

0.05 mg

195 USD