Human DnaJ (Hsp40) homolog, subfamily C, member 19 ELISA Kit | DNAJC19 elisa kit

Human Mitochondrial import inner membrane translocase subunit TIM14, DNAJC19 ELISA Kit

Cat. Num. AAA921712

• Gene Names: DNAJC19; PAM18; TIM14; TIMM14
• Reactivity: Human
• Synonyms: DnaJ (Hsp40) homolog; subfamily C; member 19; Human Mitochondrial import inner membrane translocase subunit TIM14; DNAJC19 ELISA Kit; Human Mitochondrial import inner membrane translocase subunit TIM14 (DNAJC19) ELISA kit; TIM14; TIMM14; DnaJ homolog; homolog of yeast TIM14; DNAJC19 elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of human DNAJC19. No significant cross-reactivity or interference between human DNAJC19 and analogues was observed.
• Sequence Length: 91

• Samples: Serum, plasma, tissue homogenates, Cell lysates
• Assay Type: Sandwich
• Detection Range: 28 pg/ml -1800 pg/ml.
• Sensitivity: The minimum detectable dose of human DNAJC19 is typically less than 7 pg/ml.The sensitivity of this assay, or Lower Limit of Detection (LLD) was defined as the lowest protein concentration that could be differentiated from zero. It was determined the mean O.D value of 20 replicates of the zero standard added by their three standard deviations.
• Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess.
• Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.
• Preparation and Storage: Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for DNAJC19 elisa kit

Principle of the assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for DNAJC19 has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any DNAJC19 present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for DNAJC19 is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of DNAJC19 bound in the initial step. The color development is stopped and the intensity of the color is measured.

NCBI and Uniprot Product Information

• NCBI GI #: 298231213
• NCBI GeneID: 131118
• NCBI Accession #: NP_001177162.1
• NCBI GenBank Nucleotide #: NM_001190233.1
• UniProt Accession #: Q96DA6; B2R4B1; C9JBV1
• Molecular Weight: 12,499 Da
• NCBI Official Full Name: mitochondrial import inner membrane translocase subunit TIM14 isoform 2
• NCBI Official Synonym Full Names: DnaJ (Hsp40) homolog, subfamily C, member 19
• NCBI Official Symbol: DNAJC19
• NCBI Official Synonym Symbols: PAM18; TIM14; TIMM14
• NCBI Protein Information: mitochondrial import inner membrane translocase subunit TIM14; homolog of yeast TIM14
• UniProt Protein Name: Mitochondrial import inner membrane translocase subunit TIM14
• UniProt Gene Name: DNAJC19
• UniProt Synonym Gene Names: TIM14; TIMM14
• UniProt Entry Name: TIM14_HUMAN

NCBI Description

The protein encoded by this gene is thought to be part of a complex involved in the ATP-dependent transport of transit peptide-containing proteins from the inner cell membrane to the mitochondrial matrix. Defects in this gene are a cause of 3-methylglutaconic aciduria type 5 (MGA5), also known as dilated cardiomyopathy with ataxia (DCMA). Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1, 2, 6, 10, 14 and 19. [provided by RefSeq, Jan 2012]

Uniprot Description

DNAJC19: Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity. Defects in DNAJC19 are the cause of 3-methylglutaconic aciduria type 5 (MGA5); also known as dilated cardiomyopathy with ataxia (DCMA). MGA5 is an autosomal recessive disorder characterized by early-onset dilated cardiomyopathy, growth failure, cerebellar ataxia causing significant motor delays, testicular dysgenesis, growth failure, and significant increases in urine organic acids, particularly 3-methylglutaconic acid and 3-methylglutaric acid. Belongs to the TIM14 family.

Protein type: Chaperone; Mitochondrial; Membrane protein, integral

Chromosomal Location of Human Ortholog: 3q26.33

Cellular Component: protein complex; mitochondrion; mitochondrial inner membrane; integral to membrane

Molecular Function: protein binding

Biological Process: genitalia development; cellular protein metabolic process; visual perception; protein folding; protein targeting to mitochondrion

Disease: 3-methylglutaconic Aciduria, Type V