catalog number :
MBS2602741
products type :
ELISA Kit
products full name :
Human Progressive ankylosis protein homolog, ANKH ELISA Kit
products short name :
Progressive ankylosis protein homolog, ANKH
other names :
progressive ankylosis protein homolog; Progressive ankylosis protein homolog; progressive ankylosis protein homolog; ankylosis, progressive homolog; ANKH inorganic pyrophosphate transport regulator
products gene name :
ANKH
other gene names :
ANKH; ANKH; ANK; CMDJ; HANK; MANK; CCAL2; CPPDD; KIAA1581; UNQ241/PRO274; ANK
uniprot entry name :
ANKH_HUMAN
specificity :
No cross-reaction with other factors.
storage stability :
Store all reagents at 2-8 degree C.
other info1 :
Samples: Human s erum, plasma or cell culture supernatant and organizations in the natural and recombinant ANKH concentration. Assay Type: Sandwich. Detection Range: 20 ng/ml-0.312 ng/ml. Sensitivity: Up to 0.06 ng/ml.
other info2 :
Intra-assay Precision: <= 8%. Inter-assay Precision: <= 12%
products description :
Principle of the Assay: This experiment use double-sandwich elisa technique and the ELISA Kit provided is typical. The pre-coated antibody is human ANKH monoclonal antibody and the detecting antibody is polyclonal antibody with biotin labeled. Samples and biotin labeling antibody are added into ELISA plate wells and washed out with PBS or TBS. Then Avidin-peroxidase conjugates are added to ELISA wells in order; Use TMB substrate for coloring after reactant thoroughly washed out by PBS or TBS. TMB turns into blue in peroxidase catalytic and finally turns into yellow under the action of acid. The color depth and the testing factors in samples are positively correlated.
ncbi acc num :
NP_473368.1
ncbi gb acc num :
NM_054027.4
ncbi mol weight :
54,241 Da
ncbi summary :
This gene encodes a multipass transmembrane protein that is expressed in joints and other tissues and controls pyrophosphate levels in cultured cells. Progressive ankylosis-mediated control of pyrophosphate levels has been suggested as a possible mechanism regulating tissue calcification and susceptibility to arthritis in higher animals. Mutations in this gene have been associated with autosomal dominant craniometaphyseal dysplasia. [provided by RefSeq, Jul 2008]
uniprot summary :
ANKH: Regulates intra- and extracellular levels of inorganic pyrophosphate (PPi), probably functioning as PPi transporter. Defects in ANKH are the cause of chondrocalcinosis 2 (CCAL2). Chondrocalcinosis is a common cause of joint pain and arthritis caused by calcium deposition in articular cartilage and the presence of calcium hypophosphate crystals in synovial fluid, cartilage and periarticular soft tissue. CCAL2 inheritance is autosomal dominant. Defects in ANKH are the cause of craniometaphyseal dysplasia Jackson type (CMDJ). CMDJ is a rare autosomal dominant skeletal disorder characterized by abnormal bone formation and mineralization in membranous as well as endochondral bones. Progressive thickening of the bones can cause narrowing of cranial foramina and can lead to severe visual and neurological impairment, such as facial palsy and deafness. Belongs to the ANKH family. Protein type: Membrane protein, integral; Membrane protein, multi-pass; Transporter. Chromosomal Location of Human Ortholog: 5p15.1. Cellular Component: integral to plasma membrane; outer membrane; integral to membrane; plasma membrane. Molecular Function: phosphate transmembrane transporter activity; inorganic diphosphate transmembrane transporter activity; inorganic phosphate transmembrane transporter activity. Biological Process: locomotory behavior; regulation of bone mineralization; skeletal development; inorganic diphosphate transport. Disease: Craniometaphyseal Dysplasia, Autosomal Dominant; Chondrocalcinosis 2