Human Pentosidine (PTD) ELISA Kit | MyBioSource MBS268246 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Pentosidine (PTD) ELISA Kit

catalog :

MBS268246

quantity :

48-Strip-Wells

price :

275 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS268246

products type :

ELISA Kit

products full name :

Human Pentosidine (PTD) ELISA Kit

products short name :

Pentosidine (PTD)

other names :

PREDICTED: mitochondrial chaperone BCS1 isoform X4; Mitochondrial chaperone BCS1; mitochondrial chaperone BCS1; h-BCS1; BCS1-like protein; mitochondrial complex III assembly; BC1 (ubiquinol-cytochrome c reductase) synthesis-like; BCS1-like protein

products gene name :

PTD

other gene names :

BCS1L; BCS1L; BCS; BJS; PTD; BCS1; FLNMS; h-BCS; MC3DN1; GRACILE; Hs.6719; BCS1; h-BCS1

uniprot entry name :

BCS1_HUMAN

reactivity :

Human

sequence length :

419

specificity :

No cross-reaction with other factors.

storage stability :

Store all reagents at 2-8 degree C.

other info1 :

Samples: Human serum, plasma or Cell Culture Supernatant and organizations in the natural and recombinant PTD concentration. Assay Type: Sandwich. Detection Range: 200 ng/ml-3.12 ng/ml. Sensitivity: 0.6 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 PTD 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 gi num :

578804710

ncbi acc num :

XP_006712741.1

ncbi gb acc num :

XM_006712678.1

ncbi mol weight :

47,534 Da

ncbi pathways :

Metabolism Of Proteins Pathway (106230); Mitochondrial Protein Import Pathway (576261)

ncbi summary :

This gene encodes a homolog of the S. cerevisiae bcs1 protein which is involved in the assembly of complex III of the mitochondrial respiratory chain. The encoded protein does not contain a mitochondrial targeting sequence but experimental studies confirm that it is imported into mitochondria. Mutations in this gene are associated with mitochondrial complex III deficiency and the GRACILE syndrome. Five alternatively spliced transcripts encoding the same protein have been described. [provided by RefSeq, Mar 2012]

uniprot summary :

BCS1L: Chaperone necessary for the assembly of mitochondrial respiratory chain complex III. Plays an important role in the maintenance of mitochondrial tubular networks, respiratory chain assembly and formation of the LETM1 complex. Defects in BCS1L are the cause of GRACILE syndrome (GRACILE). GRACILE stands for growth retardation, aminoaciduria, cholestasis, iron overload, lactic acidosis, and early death . It is a recessively inherited lethal disease characterized by fetal growth retardation, lactic acidosis, aminoaciduria, cholestasis, and abnormalities in iron metabolism. Defects in BCS1L are a cause of mitochondrial complex III deficiency (MT-C3D). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. Defects in BCS1L are the cause of Bjoernstad syndrome (BJS). BJS is an autosomal recessive condition characterized by sensorineural hearing loss and pili torti. The hearing loss in BJS is congenital and of variable severity. Pili torti (twisted hairs), a condition in which the hair shafts are flattened at irregular intervals and twisted 180 degrees from the normal axis, making the hair extremely brittle, is usually recognized early in childhood. Belongs to the AAA ATPase family. BCS1 subfamily. Protein type: Mitochondrial; Membrane protein, integral; Chaperone. Chromosomal Location of Human Ortholog: 2q33. Cellular Component: mitochondrion; mitochondrial respiratory chain complex III. Molecular Function: protein binding; ATP binding. Biological Process: mitochondrial respiratory chain complex I assembly; mitochondrion organization and biogenesis; mitochondrial respiratory chain complex IV assembly. Disease: Leigh Syndrome; Gracile Syndrome; Bjornstad Syndrome; Mitochondrial Complex Iii Deficiency, Nuclear Type 1

size1 :

48-Strip-Wells

price1 :

275 USD

size2 :

96-Strip-Wells

price2 :

420

more info or order :

MyBioSource product webpage