Human Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial, ALDH4A1 ELISA Kit | MyBioSource MBS9330708 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial, ALDH4A1 ELISA Kit

catalog :

MBS9330708

quantity :

48-Strip-Wells

price :

470 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS9330708

products type :

ELISA Kit

products full name :

Human Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial, ALDH4A1 ELISA Kit

products short name :

[aldehyde dehydrogenase 4 family, member A1]

products name syn :

[Human Delta-1-pyrroline-5-carboxylate dehydrogenase; mitochondrial (ALDH4A1) ELISA kit; RP11-128M10.1; ALDH4; DKFZp779M035; P5CD; P5CDh; P5C dehydrogenase; aldehyde dehydrogenase 4A1; mitochondrial delta-1-pyrroline 5-carboxylate dehydrogenase; aldehyde dehydrogenase 4 family; member A1]

other names :

[delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial isoform b; Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial; delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial; P5C dehydrogenase; aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenase; mitochondrial delta-1-pyrroline 5-carboxylate dehydrogenase; aldehyde dehydrogenase 4 family, member A1; Aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenase]

products gene name :

[ALDH4A1]

other gene names :

[ALDH4A1; ALDH4A1; P5CD; ALDH4; P5CDh; ALDH4; P5CDH; P5C dehydrogenase]

uniprot entry name :

AL4A1_HUMAN

reactivity :

Human

specificity :

No significant cross-reactivity or interference between this analyte and analogues is observed.

storage stability :

Store all reagents at 2-8 degree C

other info1 :

Samples: Undiluted original Human body fluids, tissue homogenates, secretions or feces samples. Assay Type: Sandwich (Quantitative). Detection Range: 0.625 ng/ml - 20 ng/ml. Sensitivity: 0.1 ng/ml.

other info2 :

Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].

products description :

Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of ALDH4A1 (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

ncbi gi num :

238859541

ncbi acc num :

NP_001154976.1

ncbi gb acc num :

NM_001161504.1

uniprot acc num :

P30038

ncbi mol weight :

61,719 Da

ncbi pathways :

4-hydroxyproline Degradation I Pathway (142337); Alanine, Aspartate And Glutamate Metabolism Pathway (101142); Alanine, Aspartate And Glutamate Metabolism Pathway (100063); Arginine And Proline Metabolism Pathway (82957); Arginine And Proline Metabolism Pathway (323); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Proline Catabolism Pathway (106178); Arginine Degradation I (arginase Pathway) (142142); Arginine Degradation I (arginase Pathway) (138167)

ncbi summary :

This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase which catalyzes the second step of the proline degradation pathway, converting pyrroline-5-carboxylate to glutamate. Deficiency of this enzyme is associated with type II hyperprolinemia, an autosomal recessive disorder characterized by accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. [provided by RefSeq, Jun 2009]

uniprot summary :

ALDH4A1: Irreversible conversion of delta-1-pyrroline-5- carboxylate (P5C), derived either from proline or ornithine, to glutamate. This is a necessary step in the pathway interconnecting the urea and tricarboxylic acid cycles. The preferred substrate is glutamic gamma-semialdehyde, other substrates include succinic, glutaric and adipic semialdehydes. Defects in ALDH4A1 are the cause of hyperprolinemia type 2 (HP-2). HP-2 is characterized by the accumulation of delta-1-pyrroline-5-carboxylate (P5C) and proline. The disorder may be causally related to neurologic manifestations, including seizures and mental retardation. Belongs to the aldehyde dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Amino Acid Metabolism - alanine, aspartate and glutamate; Mitochondrial; Amino Acid Metabolism - arginine and proline; Oxidoreductase; EC 1.2.1.88. Chromosomal Location of Human Ortholog: 1p36. Cellular Component: mitochondrial matrix. Molecular Function: identical protein binding; aldehyde dehydrogenase (NAD) activity; electron carrier activity; 1-pyrroline-5-carboxylate dehydrogenase activity. Biological Process: proline catabolic process; 4-hydroxyproline catabolic process; proline biosynthetic process; glutamate biosynthetic process; proline catabolic process to glutamate; proline metabolic process. Disease: Hyperprolinemia, Type Ii

size1 :

48-Strip-Wells

price1 :

470 USD

size2 :

96-Strip-Wells

price2 :

680

size3 :

5x96-Strip-Wells

price3 :

3100

size4 :

10x96-Strip-Wells

price4 :

6095

more info or order :

MyBioSource product webpage