OSTM1 Antibody (PACO11028)
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주문- SKU:
- PACO11028
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | OSTM1 Antibody (PACO11028) |
Antibody SKU: | PACO11028 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB |
Recommended Dilutions: | |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: | Human OSTM1 |
Form: | Liquid |
Storage Buffer: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
Purification Method: | Antigen Affinity purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Synonyms: | osteopetrosis associated transmembrane protein 1;OSTM1;GIPN;GL;HSPC019;OPTB5 ; |
UniProt Protein Function: | 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 adulthood. 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. |
UniProt Protein Details: | Protein type:EC 6.3.2.-; Ligase; Membrane protein, integral; Ubiquitin conjugating system; Ubiquitin ligase Chromosomal Location of Human Ortholog: 6q21 Cellular Component: lysosomal membrane Disease: Osteopetrosis, Autosomal Recessive 5 |
NCBI Summary: | 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] |
UniProt Code: | Q86WC4 |
NCBI GenInfo Identifier: | 51316434 |
NCBI Gene ID: | 28962 |
NCBI Accession: | Q86WC4.1 |
UniProt Secondary Accession: | Q86WC4,Q5R391, Q6PCA7, Q7RTW6, Q8NC29, Q8TC82, Q9Y2S9 E1P5E3, |
UniProt Related Accession: | Q86WC4 |
Molecular Weight: | 37kDa |
NCBI Full Name: | Osteopetrosis-associated transmembrane protein 1 |
NCBI Synonym Full Names: | osteopetrosis associated transmembrane protein 1 |
NCBI Official Symbol: | OSTM1 |
NCBI Official Synonym Symbols: | GL; GIPN; OPTB5; HSPC019 |
NCBI Protein Information: | osteopetrosis-associated transmembrane protein 1 |
UniProt Protein Name: | Osteopetrosis-associated transmembrane protein 1 |
UniProt Synonym Protein Names: | Chloride channel 7 beta subunit |
Protein Family: | Osteopetrosis-associated transmembrane protein |
UniProt Gene Name: | OSTM1 |