| ALDH4A1

Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial is an enzyme that is encoded by the ALDH4A1 gene.This protein belongs to the aldehyde dehydrogenase family of proteins. This enzyme is a mitochondrial matrix NAD-dependent dehydrogenase that 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. Two transcript variants encoding the same protein have been identified for this gene.
 

| Typical data

As the OD values of the standard curve may vary according to the conditions of the actual assay performance (e.g. operator, pipetting technique, wash technique or temperature effects), the operator should establish a standard curve for each test. Typical standard curve and data are provided below for reference only.

 

Concentration (pg/mL)	1000	500	250	125	62.5	31.2	15.6	0
OD	2.47	1.91	1.34	0.87	0.61	0.42	0.31	0.09
Corrected OD	2.38	1.82	1.25	0.78	0.52	0.33	0.22	-

 

| Repeatability

Intra-assay Precision (Precision within an assay)：CV% < 10%; Inter-assay Precision (Precision between assays)：CV% < 10%
 

| Recovery

The recovery of Human ALDH4A1 spiked at three different levels in samples throughout the range of the assay was evaluated in various matrices.

 

Sample Type	Range(%)	Average Recovery(%)
Serum(n=8)	84-101	96
Plasma(n=8)	92-105	102
Cell Culture Supernates(n=8)	96-109	105

 

| Linearity

Samples were spiked with high concentrations of Human ALDH4A1 and diluted with Universal Diluent to produce samples with values within the range of the assay.

 

		Serum(n=4)	Plasma(n=4)	Cell Culture Supernates(n=4)
1 : 2	Range (%)	84-95	88-96	90-110
	Average (%)	91	93	96
1 : 4	Range (%)	89-103	87-108	105-115
	Average (%)	94	98	108