Osteopetrosis Associated Transmembrane Protein 1, Recombinant Protein

GL; GIPN; Chloride channel 7 beta subunit

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

E Coli

>90%

Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.
Stability Test: The thermal stability is described by the loss rate of the target protein. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed. The loss of this protein is less than 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of OSTM1 recombinant protein 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.

SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)

18.6kDa

osteopetrosis associated transmembrane protein 1

osteopetrosis-associated transmembrane protein 1

Osteopetrosis-associated transmembrane protein 1

GL  [Similar Products]

OSTM1_HUMAN

This gene encodes a protein that may be involved in the degradation of G proteins via the ubiquitin-dependent proteasome pathway. The encoded protein binds to members of subfamily A of the regulator of the G-protein signaling (RGS) family through an N-terminal leucine-rich region. This protein also has a central RING finger-like domain and E3 ubiquitin ligase activity. This protein is highly conserved from flies to humans. Defects in this gene may cause the autosomal recessive, infantile malignant form of osteopetrosis. [provided by RefSeq, Jul 2008]

OSTM1: Required for osteoclast and melanocyte maturation and function. Defects in OSTM1 are the cause of osteopetrosis autosomal recessive type 5 (OPTB5); also called infantile malignant osteopetrosis 3. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or *****hood. OPTB5 patients manifest primary central nervous system involvement in addition to the classical stigmata of severe bone sclerosis, growth failure, anemia, thrombocytopenia and visual impairment with optic atrophy.

Protein type: Membrane protein, integral; Ligase; EC 6.3.2.-; Ubiquitin conjugating system; Ubiquitin ligase

Chromosomal Location of Human Ortholog: 6q21

Cellular Component: cytoplasm; cytosol; integral to membrane; intracellular membrane-bound organelle; lysosomal membrane; nucleus

Biological Process: osteoclast differentiation; transmembrane transport

Disease: Osteopetrosis, Autosomal Recessive 5

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